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Douglas T. Cromack, MD

  • Assistant Professor of Orthopedic Surgery
  • Division of Plastic and Reconstructive Surgery
  • University of Texas Health Science Center at San Antonio
  • San Antonio, Texas

After recovery medicine 93 948 safe triamcinolone 4 mg, his examination demonstrated generalized chorea medicine qd triamcinolone 4mg online, past-pointing and dysarthria treatment jiggers generic 4 mg triamcinolone otc, limb and gait ataxia symptoms 7 days after implantation 4mg triamcinolone for sale, and impaired vertical gaze eye movements symptoms quivering lips order triamcinolone 4 mg free shipping. An important initial step in the evaluation of this clinical scenario is to distinguish between a progressive psychomotor decline treatment goals for ptsd buy 4 mg triamcinolone, as in this case symptoms 6 days before period discount 4mg triamcinolone fast delivery, and a static encephalopathy medications that cause high blood pressure triamcinolone 4 mg. Static encephalopathies can be broadly classified into antenatal insults (infections [cytomegalovirus, herpes simplex virus, rubella], toxins [alcohol, cocaine]) and perinatal (hypoxic-ischemic encephalopathy, hyperbilirubinemia). It is also important to determine the point at which regression began, and the evolution of the psychomotor symptomatology; were age-appropriate milestones achieved (figure)? In this case, the patient achieved age-appropriate motor and cognitive milestones and thereafter experienced psychomotor regression. The age at onset in the second decade of life and apparent absence of family history might be consistent with an autosomal recessive condition, rather than an autosomal dominant condition. When considering a differential diagnosis for early-onset cognitive impairment, it is useful to identify associated neurologic features (figure). Many of the listed conditions may be deemed unlikely given the mode of inheritance (Huntington disease and similar disorders, spinocerebellar ataxia, dentatorubral pallidoluysian atrophy) whereas others may require specific investigation. A paraneoplastic or autoimmune disorder is most unlikely given the slow evolution of symptoms. An important finding on clinical examination was the presence of a vertical supranuclear gaze palsy. This sign narrows the differential diagnosis considerably in a patient presenting with ataxia and chorea (figure). Although not present in this patient, splenomegaly is an important clinical feature to exclude in a young patient presenting with a mixed movement disorder and a key finding in generating a differential diagnosis. Vertical supranuclear gaze palsy is an important clinical sign and invariably present in this disorder when there are neurologic manifestations beyond infancy. It is also the first neurologic sign to develop in individuals who present with organomegaly. The history also provides a useful clue of gelastic cataplexy (muscle atonia after episodes of heightened emotion). Clinical presentation, disease progression, and severity are strongly influenced by age at onset of neurologic symptoms. Presentation in early infancy is marked by delayed developmental motor milestones. Juvenile onset, as in our case, presents with gait problems, falls, clumsiness, cataplexy, and cognitive problems. Our patient was treated with levetiracetam for control of seizures and haloperidol to manage choreiform movements. Miglustat acts by reversibly inhibiting glucosylceramide synthase, which catalyzes the first step of glycosphingolipid synthesis. Finally, the pattern of neurologic system involvement (chorea, seizure, vertical gaze, palsy) narrows the differential diagnosis further. Eavan Mc Govern: acquisition of case history information, composition of case history and discussion. Timothy Counihan: critical revision of the manuscript, supervision of the case history and discussion. Clues from the history provide valuable information regarding the underlying process. Recommendations for the diagnosis and management of Niemann-Pick disease type C: an update. Miglustat in adult and juvenile patients with Niemann-Pick disease type C: long-term data from a clinical trial. Three months prior to presentation, the patient suddenly developed violent muscle jerks involving the right side of his body and face that impaired his gait and balance. Over the following weeks, he experienced fluctuating symptoms of confusion, memory impairment, insomnia, and paranoid delusions. His muscle jerks and unstable gait were intermittent with return to baseline in between attacks, but they increased in frequency and occurred many times throughout the day. He was found to be mildly hyponatremic and was eventually admitted to a psychiatric ward for treatment of acute psychosis. He was a retired mechanical engineer and was physically active prior to the onset of symptoms. He registered 3 items but was unable to recall them at 5 minutes and was unable to complete serial 7s. He had a wide-based gait with prominent right lateral pulsion and retropulsion, without any observed muscle jerks during gait examination. Occasional myoclonus involving the right side of his face and right upper extremity were observed, which were associated with loss of awareness and dystonic posturing of the right arm. Based on the history and physical examination, what is the differential diagnosis? Though the right-sided myoclonus may be cortical or subcortical, the localization can be narrowed based on other findings. Retropulsion is an extrapyramidal sign often due to loss of postural reflexes and is seen in disorders that involve the basal ganglia; the asymmetric right lateral pulsion localizes this to the left basal ganglia. The patient also displays cognitive deficits in orientation, memory, and attention, which indicate that there might be further cortical or subcortical involvement. The differential diagnosis should consider subacute encephalopathies that present with this constellation of findings. These findings are consistent with limbic encephalitis; however, other autoimmune and infectious etiologies should be ruled out. Serum autoimmune and inflammatory workup including erythrocyte sedimentation rate, C-reactive protein, antinuclear antibodies, rheumatoid factor, Sjцgren syndrome A/Sjцgren syndrome B, angiotensin-converting enzyme, antithyroid peroxidase, and antithyroglobulin were unremarkable. A paraneoplastic antibody panel (table e-1 on the Neurology Web site at Neurology. Can a diagnosis of paraneoplastic limbic encephalitis be made in the absence of cancer or a paraneoplastic antibody? Corticosteroids were not given at this time due to his diabetes, psychiatric symptoms, and availability of plasma exchange. During a follow-up visit, the patient was initially alert but became progressively drowsy and unresponsive. He was readmitted to the hospital, with concern for status epilepticus or worsening of his underlying condition. He also received levetiracetam, which required uptitration to 1,500 mg twice daily to achieve control of the myoclonus. Four months after his discharge from the hospital, he experienced almost complete resolution of symptoms, with only sporadic myoclonus associated with insomnia. Cholfin: analysis and interpretation of data, imaging interpretation, critical revision of the manuscript. Restrepo: analysis and interpretation of data, imaging interpretation, critical revision of the manuscript for important intellectual content and supervision. Limbic encephalitis is an autoimmune process affecting the medial temporal lobes or limbic structures that can present either acutely or subacutely with symptoms of confusion, memory impairment, sleep disturbance, seizures, and psychiatric disturbance. Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype. Neuropsychological course of voltage-gated potassium channel and glutamic acid decarboxylase antibody related limbic encephalitis. In addition to supporting such mundane movements, the motor system allows athletes, dancers, and musicians to utilize the very same circuitry to achieve millisecond and millimeter precision. Higher-level motor control involves the premotor and supplementary motor cortices in interaction with the basal ganglia and cerebellum. The coordinated motor plan devised by these circuits is transmitted through the corticospinal tracts to stimulate the motor fibers of peripheral nerves that activate select muscles. The motor system can be divided into the pyramidal system and the extrapyramidal system. The pyramidal system includes the corticospinal tracts that span the brain, brainstem, and spinal cord to communicate with the peripheral nervous system. The extrapyramidal system includes the basal ganglia and cerebellum, which serve to initiate, pattern, and coordinate movements. Lesions in the pyramidal system produce weakness, lesions in the cerebellum can produce impaired coordination of movements (ataxia and dysmetria), and lesions in the basal ganglia can alter muscle tone (rigidity) and cause pathologically decreased or increased movement (see "Disorders Presenting with Abnormal Movements"). Lesions affecting higher-level motor cortices impair the ability to perform complex learned motor tasks (apraxia). The pyramidal system has 2 main components: upper motor neurons in the central nervous system and lower motor neurons whose axons lie in the peripheral nervous system. The upper motor neurons begin in the precentral gyrus of the frontal lobe and travel in the corticospinal tracts through the subcortical white matter and anterior brainstem, crossing at the cervicomedullary junction to descend in the contralateral spinal cord. The axons of the corticospinal tracts synapse on lower motor neurons in the anterior horn of the spinal cord. These lower motor neurons travel through ventral roots into peripheral nerves and terminate at neuromuscular junctions to stimulate muscle contraction. Hemiparesis refers to partial weakness and hemiplegia refers to complete paralysis. Localization in disorders of the pyramidal motor system is guided by determining the distribution of weakness. As in all neurologic diagnosis, the time course guides the differential diagnosis of the cause of the lesion. Establishing which parts of the body are weak is fundamental to determining the potential localization of a lesion along the motor pathway. When the distribution of weakness includes the face, the lesion must be located at the level of the pons or higher. Unilateral weakness of the face, arm, and leg on one side localizes to the contralateral cerebral hemisphere or cerebral peduncle. Lesions at the level of the facial nucleus/nerve in the pons generally cause weakness in the ipsilateral face and contralateral body, since the facial nerves project ipsilaterally, but the corticospinal tracts have not yet crossed at this level. Weakness of only the arm and leg on one side with no facial involvement can occur due to lesions at the level of the lower medulla or cervical spinal cord, but small lesions in the cerebral hemisphere can also produce this pattern. Weakness affecting the extensors of the upper extremity more than the flexors and the lower extremity flexors more so than the extensors suggests a lesion in the central nervous system. Weakness affecting a single limb in its entirety (monoparesis or monoplegia) can be caused by a small lesion in the cerebral hemisphere, a lesion in the spinal cord, a polyradiculopathy, or a plexopathy. However, small lesions in the cerebral hemispheres can produce patterns that mimic peripheral lesions such as the "pseudo radial nerve palsy" pattern that can be caused by a small stroke in the hand region of the motor cortex. An · Lesions in the central nervous system can cause hyperreflexia, increased tone, and abnormal reflexes such as Babinski and Hoffmann signs, but these findings may not be present acutely. Several aspects of the physical examination help make this distinction: · Weakness without any sensory changes and with normal reflexes generally suggests a problem at the level of the neuromuscular junction or muscle. Cranial nerve palsies associated with motor deficits in the extremities suggest localization to the brainstem. Since nearly all cranial nerves project ipsilaterally and the corticospinal tract crosses at the cervicomedullary junction, brainstem lesions cause ipsilateral deficits in the face/eyes and contralateral deficits in the extremities. Bowel and bladder dysfunction generally implies a lesion of the spinal cord or cauda equina. The cases that follow emphasize these principles in the approach to patients with weakness. Approximately 1 year before her first visit, the patient developed difficulty walking, which caused multiple falls without serious injury. Sentence structure in her e-mails was abnormal but her family believed that her comprehension was intact. She was still able to do most of her activities of daily living, but only cooked simple meals, and had stopped driving because of a minor car accident. She also had kidney stones necessitating a total nephrectomy after failed lithotripsy, and experienced urinary incontinence and constipation. She had a family history of dementia in her mother when she was in the eighth decade of life, but no other family history of dementia or neurodegenerative illness. Further cognitive testing showed decreased naming and difficulty understanding a syntactically complex sentence. Ideomotor, limb kinetic, and oral apraxias were prominent, as were bilateral palmar grasp responses. She had severe impairment of fine finger movements and rapid alternating movements due to decreased amplitude and frequent arrests of movement. The patient was referred to a movement disorders specialist who also noted extrapyramidal signs of bradykinesia and postural instability, apraxia, and myoclonus, with apraxia being the dominant component (video). Left parietal lobe lesions, in particular, have been associated with buccofacial and bilateral limb apraxia. Cases of prion disease presenting with abnormal movements, myoclonus, aphasia, and apraxia are well described. The venereal disease research laboratory test, oligoclonal bands, myelin basic protein, cytology, and cryptococcal antigen were all negative. The lateral and third ventricles were prominent, with periventricular and subcortical T2 hyperintensities. The patient also had myoclonus, which can be best treated with trials of levetiracetam, clonazepam, or valproic acid. Question 5: What other steps should be taken in the care of a patient with incurable, advancing neurodegenerative disease? Over the course of 2 years, the patient deteri- Note marked attenuation of subcortical white matter. She became globally aphasic, and her difficulty walking progressed so that she required a wheelchair for mobility. Her examination was further marked by myoclonus in the right arm, with mild rigidity in all extremities and dystonic posturing in the left hand. While in hospice, she developed aspiration pneumonia and died 3 years after symptom onset. Autopsy revealed a 1,190-g brain with moderate frontal and parietal and mild temporal atrophy. Coronal sections revealed severe dilatation of the lateral ventricles and severe attenuation of the subcortical white matter (figure 2). Microscopically, there was severe white-matter rarefaction with loss of both axons and myelin, and frequent neuroaxonal spheroids and pigmented glia and macrophages (figure 3). Two separate neuropathologists confirmed the diagnosis of adult-onset leukodystrophy with neuroaxonal spheroids and pigmented glia. A recent literature review reported that the age at onset varies from 15 to 78 years, with a mean of 42 years of age. The duration of symptoms ranged from 2 months to 34 years, with symptoms including dementia, apraxia, ataxia, urinary incontinence, and extrapyramidal symptoms. The differential diagnosis includes frontotemporal dementia, corticobasal degeneration, and other leukoencephalopathies such as metachromatic leukodystrophy, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, and Binswanger disease. This gray-matter involvement may reflect neuronal death due to lack of sustaining cortical/subcortical projecting fibers, or may also be due to white-matter damage to tracts that traverse these nuclei. Microscopy reveals widespread leukoencephalopathy with axonal spheroids and macrophages in affected white matter. The spheroids are best identified with Bielschowsky, Bodian, and antineurofilament immunostains. Zadikoff treated the patient in this case report, provided references, and made several revisions to this case report. Bigio made the pathologic diagnosis for this patient, provided the pathologic description in the case report, provided references, and provided the pathologic figures for this case report. Gitelman treated the patient in this case report and made substantial revisions to this case report. Pressman serves on the editorial team of the Residents and Fellows Section of Neurology, and writes for About. Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. Cognitive and magnetic resonance imaging aspects of corticobasal degeneration and progressive supranuclear palsy. Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia can present as frontotemporal dementia syndrome. Adult onset leukodystrophy with neuroaxonal spheroids: clinical, neuroimaging and neuropathologic observations. The symptoms began abruptly 2 hours earlier during her daily work as a housekeeper when she suddenly noticed a "double tap" sound on each step of her right foot. She denied any history of trauma to the lumbar spine or to the affected lower extremity. Ankle and toe plantar flexion, knee flexion, as well as hip abduction, extension, and internal rotation, were normal. The Achilles tendon and patellar reflexes were elicited symmetrically (21) on both sides. Sensory examination demonstrated decreased sensation to pinprick on the dorsum of the right foot and the patient reported a vague discomfort in the lateral part of the right lower leg. She was able to walk unaided; however, she could not stand on the heel of her right foot. What is the most probable anatomic location of the lesion responsible for these symptoms? The presence of focal muscle weakness in a nonpyramidal distribution without evidence of corticospinal tract impairment. Several authors have described rare central causes of foot drop, such as lesions affecting the paracentral lobule1. Likewise, disorders of the neuromuscular junction or the muscles are usually excluded because they generally manifest with diffuse weakness affecting bulbar, proximal, or distal muscles. Therefore, foot drop is commonly attributed to lower motor neuron pathology and L5 radiculopathy is often suspected in the context of herniated nucleus pulposes or foraminal stenosis. The second most common cause is fibular (peroneal) neuropathy, particularly at the region of the knee.

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It possesses both these qualities at once symptoms 6dpiui triamcinolone 4 mg free shipping, whereas in organic cerebral In a letter from Freud to Josef Breuer medications without a script generic triamcinolone 4 mg without prescription, the following theories of hysteria are offered: the theorem concerning the constaacy of the sum of excitation; the theory of memory; and the theorem which lays it down that the contents of different states of consciousness are not associated with one another sewage treatment generic 4mg triamcinolone otc. Hysterical paralyses are much more frequently accompanied by disorders of sensibility than ai symptoms and diagnosis triamcinolone 4 mg generic. The lesion in hysterical paralyses consists in nothing other than the inaccessibility of the 1950A 1/184 organ or function concerned to the associations of the conscious ego medications like zovirax and valtrex 4mg triamcinolone otc. This purely functional alteration is caused by the fixation of this conception in a subconscious association with the memory of the trauma; and this conception does not become liberated and accessible so long as the quota of affect of the psychical trauma has not been eliminated by an adequate motor reaction or by conscious psychical activity symptoms of hiv discount triamcinolone 4 mg free shipping. The etiological factors include: exhaustion owing to abnormal satisfaction medicine 2015 song discount triamcinolone 4mg fast delivery, inhibition of the sexual function medicine on airplane order triamcinolone 4 mg without a prescription, affects accompanying these practices, and sexual traumas before the age of understanding. Wilhelm Fliess, a man 2 years younger than Freud, was a nose and throat specialist living in Berlin with 1/179 Draft B. Fliess was a man of great ability, with very wide interests in general biology; but he pursued theories in that field which are regarded today as eccentric and quite untenable. Its source is masturbation, the frequency of which runs completely parallel with the frequency of male neurasthenia. Girls are sound and not neurasthenic; and this is true as well of young married women, in spite of all of papers which presented organized accounts of his developing views. The material in these drafts and letters was not intended by their author the sexual traumas of this period of life. In comparatively rare cases neurasthenia appears in married wome and in older unmarried ones in its pure form; it is then as the considered expression of his opinions, and it is often framed in a highly condensed form. The mixed neurosis of women is derived from neurasthenia in men in all those not infrequent cases in which the man, being a sexual neurasthenic, suffers from impaired potency. The admixture of hysteria results directly from the holding back of the excitation of the act. Every case of neurasthenia is marked by a certain lowering of self confidence, by pessimistic expectation and an inclina- Some of the problems in the study of hysteria are presented. They include: Is the anxiety of anxiety neuroses derived from the inhibition of the sexual function or from the anxiety linked with their etiology? To what extent does a healthy person respond to later sexual traumas differently from an unhealthy one? Is there an innate neurasthenia with innate sexual weakness or is it always acquired in youth? The theses include: 1) no neurasthenia or analogous tion to distressing antithetic ideas. Anxiety neurosis appears in two forms: as a chronic state and as an attack of anxiety. The chronic symptoms are: 1) anxiety relating to the body (hypochondria); 2) anxiety relating to the functioning of the body (agoraphobia, claustrophobia, giddiness on heights); and 3) anxiety relating to decisions and memory. Periodic depression, an attack of anxiety lasting for weeks or months, is another form of anxiety neurosis. It is concluded that the neuroses are entirely preventable as well as entirely incurable. The following groups were proposed for observation: men and women who have 1/184 Letter 14. Four new cases are discussed whose etiology, as shown by the chronological data, was given as coitus interruptus. How anxiety originates (1894) the anxiety of the neurotic patient has a great deal to do with sexuality; and in particular, with coitus interruptus. Anxiety neurosis affects women who are anes- Her diagnosis was that of simple anxiety neurosis. She was described as a nice, stupid young woman in whom the anxiety was highly developed. The fourth case is a 34-year-old man who was (without having masturbated) only moderately potent. Included in the morphology of the neuroses are the following categories: neurasthenia and the pseudoneurasthenias, anxiety neurosis, obsessional neurosis, hysteria, melancholia and mania, the mixed neuroses, and the ramifications of the neuroses and transitions to the normal. The etiology of the neuroses covers the following neuroses: neurasthenia, anxiety neurosis, obsessional neurosis and hysteria, melancholia, and the mixed neuroses. It also covers: the basic etiological formula, the sexual factors in their etiological significance, an examination of the patients, objections thetic in coitus just as much as sensitive ones. Several cases in which anxiety arose from a sexual cause are presented: anxiety in viiginal people; anxiety in intentionally abstinent people; anxiety of necessarily abstinent people; anxiety of women living in coitus interruptus; anxiety of men practicing coitus interruptus; anxiety of men who go beyond their desire or strength; and anxiety of men who abstain on occasion. In anxiety neurosis there must be a deficit to be noted in sexual affect, in psychical libido. If this connection is put before women patients, they are always indignant and declare that on the contrary they now have no desire whatever. Men patients often confirm it as an observation that since suffering from anxiety they have felt no sexual desire. When there is an abundant development of physical sexual tension, but this cannot and proofs, and the behavior of asexual people. The following points were to be included in a discussion of theory: the points of contact with the theory of constancy, the sexual process in the light of the theory of constancy, the mechanism of the neuroses, the parallel between the neuroses of sexuality and neuroses. A part is played in this by the accumulation of physical tension and the prevention of discharge in the psychical direction. There is a kind of conversion in anxiety neurosis just as there is in hysteria; but in hysteria it is psychical excitation that takes a wrong path exclusively into the somatic field, whereas here it is a physical tension, which cannot enter the psychical field and therefore remains on the physical path. Three mechanisms of the neuroses are presented: transformation of affect (conversion hysteria), displace- Two cases are presented with a discussion of each one: In the first case, that of a 27-year-old man, there was a hereditary disposition: his father suffered from melancholia and his sister had a typical anxiety neurosis. In every case what seems to undergo these alterations is sexual excitation, but the impetus to them is not, in every case, something sexual. In every case in which neuroses are acquired, they are acquired owing to disturbances of sexual life; the preparations for using a condom were enougn to make him feel that the whole act was something forced on him and his enjoyment of it something he was persuaded into. The fear of infection and the decision to but there are people in whom the behavior of their sexual affects is disturbed hereditarily, and they develop use a condom laid the foundation for what has been described as the factor of alienation between the somatic the corresponding forms of hereditary neuroses. The subject brought psychical sexual weakness on himself because he spoiled coitus for himself, and his physical health and 15 1950A 1/214 production of sexual stimuli being unimpaired, the situation gave rise to the generation of anxiety. This was paranoia stands alongside of obsessional insanity as an intellectual psychosis. Paranoia, in its classical form, is a pathological mode of defense, like hysteria, obsessional neurosis and hanucinatory confusion. People become paranoiac over things that they cannot put up with, provided that they possess the peculiar psychical disposition for it. The purpose of paranoia is to fend off an idea described as a mild but very characteristic case of depression, melancholia. The symptoms, apathy, inhibition, intracranial pressure, dyspepsia, and insomnia complete the picture. It is a question of an abuse of a psychical mechanism which is very commonly employed 1/199 Letter 21. The first, a 34-year-old physician, suffered for many years from organic sensitivity of the eyes: phospheum (flashes), dazzle, scotomas, etc. This was diagnosed as a typical case of hypochondria in a particular organ in a masturbater at periods of sexual excitation. This normally operating mechanism of substitution is abused in obsessional ideas for purposes of defense. The delusional idea is maintained with the same energy with which another, intolerably distressing, idea is fended off from the ego. Paranoia and hallucinatory confusion are the two psychoses of defense or contrariness. He alleged that his potency has been capricious from the first; admitted masturbation, but not too prolonged; and had a period of abstinence behind him now. Melancholia is generated as an intensification of neurasthenia through masturbation. The type and extreme form of melancholia seems to be the periodic or cyclical hereditary form. Potent individuals easily acquire anxiety neuroses; impotent ones incline to melancholia. A description of the effects of melancholia includes: psychical inhibition with instinctual impoverishment and pain concerning it. Anesthesia seems tu encourage melancholia; however, anesthesia is not the cause of melancholia but a sign of disposition to it. There is an interval of hours or days between the instigation and the outbreak of the symptoms. Even without an instigation there is an impression that there must be an accumulating stimulus which is present in the smallest quantity at the beginning of the interval and in the largest quantity towards its end. In psychiatry, delusional ideas stand alongside of obsessional ideas as purely intellectual disorders, and be a matter of summation, in which susceptibility to etiological factors lies in the height of the level of the stimulus already present. Migraine has a complicated 16 455-582 0 - 72 2 1950A 1/215 etiology, perhaps on the pattern of a chain etiology, where approximate cause can be produced by a number of factors directly and indirectly, or on the pattern of a summation etiology, where, alongside of a specific cause, apart, however, from the rest of them, and constitutes such a formidable and self contained entity, that it was printed in a detached shape at the end of the first volume of the Standard Edition. One letter, Number 39, written on January 1,1896, is so closely connected with the Project that it too has been removed from its proper place in the correspondence and printed as an appendix to the Project. That Freud had also during all this period been concerned with clinical matters as well, is conclusively shown by the fact that on the very same day on which he dispatched this letter, he also sent Fliess Draft K. Migraines appear rarest in healthy males; are restricted to the sexual time of life; and appear frequently in people with disturbed sexual discharge. Migraine can be produced by chemical stimuli too, such as: human toxic emanations, scirocco, fatigue, and smells. Therefore, it is not impossible that migraine may include a spastic innervation of the muscles of blood vessels in the reflex sphere of the dural region. Her husband, a commercial traveller, had had to leave her a few weeks after their marriage. To pass the time, she was sitting at the piano singing, when suddenly she felt ill in her abdomen the neuroses of defense are discussed. A comparison is made between 3 different emotional states: hysteria, obsessional neurosis, and one form of paranoia. They are all pathological aberrations of normal psychical affective states: of conflict (hysteria), of self-reproach (obsessional neurosis), of mortificatidn (paranoia), of mourning (acute hallucinatory amentia). They differ from these affects in and stomach, her head swam, she had feelings of oppression and anxiety and cardiac paraesthesia; she thought she was going mad. Next day, the servant told her that a woman living in the same house had gone mad. From that time on she was never free of an obsession, accompanied by anxiety, that she was going to go mad too. It was assumed that her condition then had been an anxiety attack: a release of sexual feeling which was transformed into anxiety. With probing, it was discovered that she had had another attack 4 years earlier, also while she was singing. An effort was made to determine the other ideas present in order to account for the release of sexual feeling and the fright. However, instead of revealing these intermediate links, she discussed her motives. Heredity is a precondition, in that it facilitates and increases the pathological affect. It is this precondition which mainly makes possible the gradations between the normal and extreme case. There is a normal trend toward defense; however, the trend toward defense becomes detrimental if it is directed against ideas which are also able, in the form of memories, to release fresh unpleasure, as is the case with sexual ideas. The course taken by the illness in neuroses of repression is almost always the same: 1) there is a sexual experience which is traumatic and premature and becomes repressed. During the whole of the latter part of the year 1895 stage of successful defense, which is equivalent to health except for the existence of the primary symptom. His reflections finally led to the uncompleted work which was named a Project for a Scientific Psychology. This was written in September and October, 1895, and should appear, chronoloeically, whelmed, or of recovery with a malformation. In paranoia, the primary experience seems to be of a similar nature to that in obsessional neurosis; repression occurs after the memory at this point in the Fliess papers. The periods between 8 to 10 and 13 to 17 are the transitional periods during which repression occurs. A thesis is presented that memory is present not once but several times over, that it is laid down in various species of indications. The different registrations are also the scenes for hysteria occur in the first period of childhood (up to 4 years), in which the mnemic residues are not translated into verbal images. The scenes for paranoia fall in the prepubertal period and are aroused in maturity. Hysteria is the only neurosis in which symptoms are possible even without defense. Consciousness, as regards memories, consists of the verbal consciousness pertaining to them; that is, in access to the associated word presentations. Consciousness is not attached exclusively and inseparably either to the so-called unconscious or to the so-called conscious realm, so that these names seem to call for rejection. Consciousness is determined by a separated (not necessarily topographically) according to the neurones which are their vehicles. The perceptions are neurones in which perceptions originate, to which consciousness attaches, but which in themselves retain no trace of what has happened. The indication of perception is the first registration of the perceptions; it is quite incapable of consciousness, and is arranged according to associations by simultaneity. Unconsciousness is the second registration, arranged according to other (perhaps causal) relations. The unconsciousness traces correspond to conceptual memories; equally inaccessible to consciousness. Preconsciousness is the third transcription, attached to word presentations and corresponding to the official ego. The cathexes proceeding from preconsciousness become conscious according to *certain rules; and this secondary thought consciousness is subsequent in time, and is probably linked to the hallucinatory activation of word presentations, so that compromise between the different psychical powers which come into conflict with one another when repressions occur. These powers include: the inherent quantitative strength of a presentation and a freely displaceable attention which is attracted according to certain rules and repelled in accordance with the rule of defense. It is in the conflict between uninhibited and thought-inhibited psychical processes that symptoms arise. One species of psychical disturbance arises if the power of the uninhibited processes increases; another if the force of the thought inhibition relaxes. The successive registrations represent the psychical achievement of successive epochs of life. At the frontier between 2 such epochs a translation of the psychical material must take place. There are 3 groups of sexual psychoneuroses: hysteria, obsessional neurosis, and paranoia. On the day of the funeral I was kept waiting there and therefore reached the house of mourning rather late. At that time my family was displeased with me because I had arranged for the funeral to be quiet and simple, which they later agreed the case of hysteria between the ages of Ph and 4, of obsessional neurosis between 4 and 8, and of paranoia between 8 and 14. It is possible that the abuse may date back so far that these fs 1950A 1/242 experiences lie concealed behind the later ones. They are explained not by the functioning of erotogenic zones which have later been abandoned, but by the operation of erotogenic sensations which lose this force later. The principal sense in animals is that of smell, which has lost that position in human beings. The point that escaped me in the solution of hysteria lies in the discovery of a new source from which a new element of unconscious production arises. What I have in is dominant, hair, faces, and the whole surface of the body have a sexually exciting effect. The increase in the mind are hysterical phantasies, which regularly, as it seems to me, go back to things heard by children at an early age and only understood later. The age at which they take in information of this kind is very remarkable, from the age of 6 to 7 months onwards! The medieval theory of possession, held by the ecclesiastical courts, is said to be identical with the theory of a foreign body and a splitting of consciousness. The cruelties made it possible to understand some symptoms of hysteria which have hitherto been obscure. The complete interpretation occurred only after a lucky chance brought a fresh confirmation of paternal etiology. Thus, not only the victims but the executioners recalled in this their earliest youth. Everything goes back to the reproduction of scenes, some of which can be arrived at directly, but others always by way of phantasies set up in front of them. The phantasies are derived from things that have been heard but understood subsequently and all their material is genuine. They are protective structures, sublimations of the facts, embellishments of them, and at the same time exonerations. A second important piece of insight is that the psychical structures which, in hysteria, are affected by repression are not in reality memories, but impulses explained; the broomstick they ride on is probably the great Lord Penis. Their secret gatherings, with dancing and other amusements, can be seen any day in the streets where children play. Alongside of flying and floating on the air can be put the gymnastic feats of boys in hysterical attacks. There is a class of people, paranoiacs, who tell which arise from the primal scenes. All 3 neuroses (hysteria, obsessional neurosis, and paranoia) exhibit the stories like those of the witches. These paranoiacs complain that people put feces in their food, illtreat them at night in the most abominable way, sexually, etc. There is a distinction between delusion of memory and same elements (along with the same etiology); that is, mnemic fragments, impulses (derived from the memory) and protective fictions.

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Typically symptoms of breast cancer order 4 mg triamcinolone visa, patients follow some recommendations closely while deciding others are optional; these decisions are often made without consulting with or notifying health care professionals medicine youtube cheap triamcinolone 4mg with visa. Unintentional non-adherence is easier to remedy because it responds to patient education medicine allergy buy triamcinolone 4 mg with mastercard, simplification of treatment regimens medications contraindicated in pregnancy discount triamcinolone 4 mg otc, or the use of a reminder system medicine pictures order 4 mg triamcinolone fast delivery. Intentional non-adherence is more complex and challenging to address because patients exhibiting these behaviors often do not find evidence-based recommendations compelling medicine 66 296 white round pill order triamcinolone 4 mg with visa, lack the motivation to follow advice symptoms blood clot leg order triamcinolone 4 mg visa, or have deeply entrenched personal beliefs that conflict with health guidance medicine and health buy 4 mg triamcinolone with amex. However, chronic disease management takes place at home, with patients and their loved ones making decisions as to whether treatments should be started or continued, often without consulting the health care team. With adherence comes the idea of intentional persistence that better reflects the complexity of chronic disease management. For example, adherence rates are highest immediately before and after office visits and lowest between visits. However, a not inconsequential number of individuals never fill or pick up their first prescription, a behavior described as primary non-adherence. In one retrospective cohort study of 69,000 members of 5 large health plans, 14 ­20% of subjects with asthma were identified as having primary non-adherence. Individuals with chronic respiratory diseases may also be expected to stop smoking, attend routine ambulatory appointments (eg, cardiopulmonary rehabilitation programs), implement environmental remediation and avoidance measures, monitor air flow at home using peak flow meters or hand-held spirometers, respond to increasing symptoms using tailored action plans, gain or lose weight, and adhere to long-term oxygen therapy. Intentional non-adherence is more challenging, as it requires trust in the advice and the advisor, as well as multi-component psycho-behavioral interventions, which are discussed later. Dimensions of Adherence the World Health Organization8 identifies 5 factors that have the potential to contribute to non-adherence: socioeconomic, therapy-related, condition-related, health system­related, and patient-related factors. Illustrations of each factor are noted below, and additional examples can be found in Table 2. Socioeconomic Factors the primary socioeconomic factors influencing medication non-adherence are access to prescription benefits and out-of-pocket expenses. Without coverage, drugs are prohibitively costly for many, particularly respiratory medicines where generic drugs have not been available in the United States for years. A case in point is cost-related non-adherence that occurred following Medicare capping and increased out-of-pocket contributions. Although Part D was introduced to increase access to prescription drugs for seniors, most Medicare drug plans have a coverage gap (ie, a donut hole) that can cause significant financial distress for older patients. In addition, formulary tiers make some of the most efficacious drugs inaccessible. Affected seniors have responded to the donut hole by implementing cost-coping behaviors such as forgoing food and mortgage payments and resorting to stretching medication possession time by taking fewer doses and using prescription maintenance treatments only when symptomatic. Because these are chronic conditions, patients are expected to take medications for the rest of their lives. Most respiratory patients require multiple drugs each day; many of these drugs require more than once-daily dosing. Unique to inhaled medications, if the administration technique is inaccurate, then the drug is not delivered to the lower respiratory tract and therefore results in unintentional non-adherence. Inhaled medications also require selecting the right drug for the clinical indication (eg, quick-relief vs maintenance therapies), precisely preparing the dose (eg, priming), using the correct administration technique (eg, hand­ breath coordination, inspiratory flow) specific to the device being used (eg, pressurized metered-dose inhaler, dry powder inhaler, slow mist inhaler, nebulizer, spacers/ valved holding chambers). Incorrect beliefs about inhaled medications also lead patients to make treatment errors. If a respiratory exacerbation is not identified as resulting from non-adherence as opposed to worsening disease, then a patient may be exposed to unneeded intensification of therapies, like courses of systemic corticosteroids. Patient-Related Factors In addition to inadequate knowledge, forgetfulness, and a lack of motivation, there are other patient-related factors that may contribute to non-adherence, including mental health conditions, competing priorities (eg, food or safety), and limited health literacy. Ways to Measure Adherence Several approaches are used to measure respiratory medication adherence, each with distinct advantages and disadvantages. Prescription data are unbiased and inexpensive, but these do not confirm drug intake. Electronic monitors are objective, but widespread use has been limited by different monitors being needed for each device type. However, as industry embraces smart technology, electronic monitors embedded in each delivery device may become the norm. Most importantly, however, none of these measures currently have the ability to differentiate between intentional and unintentional non-adherence or relate disease activity back to adherence. To be done accurately, this approach would require repeated measures, which is both impractical and costly. Provider Estimate Health care providers may have a gut feeling as to whether patients are taking their medication or not. However, provider estimates have been shown to be no better than guessing in terms of distinguishing between adherent and non- adherent subjects in studies of anti-tuberculosis treatment or use of asthma controllers. This is less often because patients are devious and deceitful, but more often because patients wish to avoid confrontation, are embarrassed by their failure to follow through with medical advice, or desire to be a "good" patient. In one study, the greatest concordance between objective measures of adherence and self-report was seen when subjects admitted to non-adherence (using the inhaler less than once a day). In contrast, the greatest discordance was evident when the inhaler was reported as being used as prescribed (73% vs 15%). Moreover, 14% of subjects in this trial activated their inhalers 100 times in a 3-h period in a deliberate attempt to appear adherent, a behavior known as dumping. The reason for low medication possession rates in respiratory diseases may be due to clinicians failing to prescribe medications or due to patients failing to obtain initial prescriptions (primary non-adherence) or to refill prescriptions (secondary non-adherence). Table 3 lists common prescription possession and use measures, as well as how these are calculated. Electronic Monitors and Smart Technology Add-on transferable electronic monitors were originally developed to measure adherence for research purposes. With the advent of newer, sophisticated, so-called smart technologies, embedded and connected devices aspire to do more than just measure adherence: they can serve as reminder systems, create trackable data points for patients and clinicians to interpret, and entrain geographical positioning and air quality data to identify triggers and location of medication use. Perhaps most importantly, they seek to do what other measures of adherence have not been able to do to date, which is to connect measures of disease control with adherence. With the exception of the Doser, which is available for consumer purchase, most electronic monitors were designed to measure adherence in clinical trials. With the cost associated with smart technology no longer an impediment to wide scale production, and with Bluetooth-enabled smartphones offering data aggregation in the cloud and direct interface with electronic health platforms, pharmaceutical companies are moving ahead with embedded sensing and connectable systems in their inhaled medication delivery devices. This is expected to offer patients and clinicians the opportunity to discover personal patterns of medication use and symptoms that otherwise might not have been discernible. It also offers insurers and researchers the opportunity to learn from aggregated population health data (ie, big data). While smart devices can help monitor adherence, they will likely cost more, at least initially, than conventional unconnected inhaled delivery systems. In addition, the maximum benefit may only be derived from smart inhalers connected to the cloud, access that for many may be costprohibitive or intermittent as low-income consumers often rely on pay-as-you-go text and data-access plans. This smart future will therefore force payers to reconsider the cost of covering access in light of the value that the new devices offer in terms of supported management of certain high-risk populations. To be accepted by patients, however, these integrated devices must be intuitive to use and must provide digestible bites of actionable and relevant data when the patient wants it. Most importantly, if adherence is to be measured, patients must want to opt in and to set limits on how closely they are monitored, fearing repercussions for being identified as non-adherent. To that end, strategies to address unintentional non-adherence focus on patient education, simplification of regimens, and structures for remembering medication. Healthy People 2020 sought to increase the proportion of individuals receiving formal asthma education from 8% to 30%, but as of 2007 only 12% of adults reported having received asthma selfmanagement education. For many chronic conditions, pill boxes are used to organize medications and to serve as visual reminders of use. Innovative Strategies to Reduce Intentional Non-Adherence Despite knowledge of asthma and its management, some patients still do not find medical advice compelling or hold deeply entrenched beliefs that conflict with treatment recommendations. Interventions focused on empowering patients to set and achieve health goals of their own making is the central feature of coaching, which is also described as health or wellness coaching. Coaches typically have advanced training in behavioral sciences, with a focus on behavioral change theory, communication, and motivational skills. A final differentiating point is that, compared to patient educators who have time-limited relationships with their clients, coaches typically have ongoing, or even enduring, relationships with patients. Coaching has been shown to improve inhaler technique in adults with asthma, reduce asthma symptoms and acute health care utilization in children, and enhance quality of life of parents of asthmatic children. Originating decades ago, patient navigators and patient advocates were nurses and other highly trained lay individuals who guided cancer patients through complex payer and health care systems to obtain multiple consultations and diagnostic tests so that they could make informed decisions about their cancer treatment. The critical components of this skill set included education, support, and care coordination across the care continuum (home, acute care, primary care). Historically, medicine has taken a paternalistic approach to patient­provider relationships, with clinicians deciding what is best for the patient based on their expertise and the scientific evidence. This evolved to an informative model of care in which providers informed patients about why a treatment was recommended, later adding an interpretative approach that helped patients find their own preferences for care. Motivational interviewing is a specific microcounseling approach developed by clinical psychologists Miller and Rollnick as part of a screening, brief intervention, and referral to treatment strategy for problem drinking and drug use. In the second step (focusing), a candid conversation helps draw a connection between current behaviors and the benefits of future behavioral change. In this step, beliefs regarding the benefits and negative sequelae of their current approaches (pros and cons) are explored. This approach did not have added value above the self-management training the practices had already received83 and was not cost-effective. Factors related to disease, treatment, health care systems, medication cost and access, as well as patient-specific attributes associated with suboptimal adherence have been explicated. By differentiating intentional from unintentional non-adherence, clinicians will be better able to match intervention strategies to the underlying cause of non-adherence. A review of the literature identified novel psychotherapeutic interventions to address intentional non-adherence that, while still in development, offer clinicians some early signals as to approaches that may be successful in managing the most complex and challenging adherence issues in the future. To achieve disease control and improve patient safety, such as reduced morbidity and reduced exposure to unnecessary treatment intensification, adherence must be improved. To date, patients have borne an unequal portion of blame for non-adherence because much of non-adherence is unintentional. This support must come from the health professionals who care for them, and these health professionals must be trained in adherence and knowledgeable about effective tailored adherence interventions. Rather, it will require a team using multidimensional approaches that address all the factors that contribute to nonadherence. This is well worth doing because increasing the effectiveness of adherence interventions may have a far greater impact on the health of the population than any improvement in specific medical treatments. Longitudinal association between medication adherence and lung health in people with cystic fibrosis. Management of adherence and chronic rheumatic disease in children and adolescents. Adherence challenges in the management of type 1 diabetes in adolescents: Prevention and intervention Curr Opin Pediatr 2010;22(4):405-411. Nieuwlaat R, Wilczynski N, Navarro T, Hobson N, Jeffery R, Keepanasseril A, et al. Interventions to enhance patient adherence to medication prescriptions: Scientific review. Interventions improve poor adherence with once daily glaucoma medications in electronically monitored patients. Objective assessment of adherence to inhalers by patients with chronic obstructive pulmonary disease. Prescription fill patterns in underserved children with asthma receiving subspecialty care. Inhaled corticosteroid beliefs, complementary and alternative medicine and uncontrolled asthma in urban minority adults. Understanding different beliefs held by adherers, unintentional non-adherers, and intentional non-adherers: Application of the Necessity-Concerns Framework. Incorporating value-based insurance design to improve chronic disease management in the Medicare Advantage program 2016. Patient knows best: Blinded assessment of nonadherence with antituberculous therapy by physicians, nurses, and patients compared with urine drug levels. Prescription patterns for asthma medications in children and adolescents with health care insurance in the United States. Prescription patterns, adherence and characteristics of non-adherence in children with asthma in primary care. The digital asthma patient: the history and future of inhaler based health monitoring devices. Analysis, modelling and sensing of both physiological and environmental factors for the customized and predictive self-management of asthma. In Proc 6th Panhellenic Conf of Biomedical Technology, held May 68, 2015, in Athens, Greece. Effectiveness of population health management using the Propeller Health asthma platform: A randomized clinical trial. Vital signs: Asthma prevalence, disease characteristics, and self-management education: United States, 2001-2009. Opportunities and challenges for smartphone applications in supporting health behavior change: qualitative study. Leiva-Fernandez J, Leiva-Fernandez F, Garcнa-Ruiz A, Prados-Torґ ґ res D, Barnestein-Fonseca P. Strategies used by older adults with asthma for adherence to inhaled corticosteroids. A systematic review of the literature on health and wellness coaching: Defining a key behavioral intervention in healthcare. Inhaler device technique can be improved in older adults through tailored education: findings from a randomised controlled trial. Telephone coaching for parents of children with asthma: impact and lessons learned. A randomized controlled trial of parental asthma coaching to improve outcomes among urban minority children. Integrated interdisciplinary care for patients with chronic obstructive pulmonary disease reduces emergency department visits, admissions and costs: A quality assurance study. Feasibility, acceptability and preliminary effectiveness of patient advocates for improving asthma outcomes in adults. Clearing clinical barriers: Enhancing social support using a patient navigator for asthma care. A patient advocate to facilitate access and improve communication, care, and outcomes in adults with moderate or severe asthma: Rationale, design, and methods of a randomized controlled trial. Shared decision making and motivational interviewing: Achieving patient-centered care across the spectrum of health care problems. A randomized trial to improve patient-centered care and hypertension control in underserved primary care patients. Shared treatment decision making improves adherence and outcomes in poorly controlled asthma. Implementation of self management support for long term conditions in routine primary care settings: Cluster randomised controlled trial. Evaluation of a web-based asthma management intervention program for urban teenagers: Reaching the hard to reach. Discussion Lugogo: I struggle with how to really get at non-adherence in the clinic, and I think a tool of some kind would be nice to have. George: There are some tools that have been developed by industry as part of clinical trials that are not really available for mass market. I use the Medication Adherence Report Scale for Asthma, which correlates with both objective measures of adherence as well as pharmacy refill data. And if they admit that they missed at least 1 dose, they were likely to have missed 50% or more of their doses. I used to think that economics was the dismal science, but now it appears that behavioral nursing is. I do know that patients in the donut hole have clearly been shown to demonstrate some of the patterns we talked about yesterday: they take only their morning dose and not their evening dose, or go to every other day, or based on symptoms only. We also know that patients are more adherent immediately before and after office visits. The third thing, and I know you appreciate this, is that the interface with the doctors is important too. I think most doctors hate to get the constant reminders we get from some of these systems. George: Right, I focused on just patients here, but there is a wealth of information that we know. Payers cannot afford for non-adherence to be the driving mechanism for the addition or substitution of these drugs. This procedure would be 1% risk for hemorrhage, or what have you, and then the patient makes their choice. Giordano: Were any of the researchers able to establish a correlation between the rate of non-adherence and the number of comorbidities? George: There is clearly a correlation between the number of drugs taken per day or the number doses per day. Giordano: Maureen, in your opinion, if we want to identify in advance potential non-adherers for more intense education, in other words, create a list of usual suspects? You might expect that they would all question whether the diagnosis is right, if the treatment is needed, and if the treatment is safe. Mann: I was wondering if you could give the top 3 things for non-adherence that you should discuss with your patients. There are different models, I think of a 30-d hospitalization and they walk out the door with a smart inhaler where you can monitor the albuterol use and the maintenance use. Mann: I think for clinicians to know these are the top reasons we really need to explore with these patients would be great. George: I think a full meeting on this would be really helpful, but I may be biased.

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