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1/168. Fatal familial insomnia: a new Austrian family.

    We present clinical, pathological and molecular features of the first Austrian family with fatal familial insomnia. Detailed clinical data are available in five patients and autopsy in four patients. Age at onset of disease ranged between 20 and 60 years, and disease duration between 8 and 20 months. Severe loss of weight was an early symptom in all five patients. Four patients developed insomnia and/or autonomic dysfunction, and all five patients developed motor abnormalities. Analysis of the prion protein (PrP) gene revealed the codon 178 point mutation and methionine homozygosity at position 129. In all brains, neuropathology showed widespread cortical astrogliosis, widespread brainstem nuclei and tract degeneration, and olivary 'pseudohypertrophy' with vacuolated neurons, in addition to neuropathological features described previously, such as thalamic and olivary degeneration. Western blotting of one brain and immunocytochemistry in four brains revealed quantitative and regional dissociation between PrP(res)(the protease resistant form of PrP) deposition and histopathology. In the cerebellar cortex of one patient, PrP(res) deposits were prominent in the molecular layer and displayed a peculiar patchy and strip-like pattern with perpendicular orientation to the surface. In another patient, a single vacuolated neuron in the inferior olivary nuclei contained prominent intravacuolar granular PrP(res) deposits, resembling changes of brainstem neurons in bovine spongiform encephalopathy.
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2/168. Lamotrigine associated with insomnia.

    PURPOSE: To review the incidence of lamotrigine (LTG)-associated insomnia in an adult tertiary care epilepsy outpatient clinic. methods: The records of all patients who had received LTG were reviewed to identify patients who had experienced insomnia after introduction of this drug. patients were included if they had experienced a sleep disturbance of sufficient severity to require a discontinuation of LTG or a dose reduction. RESULTS: Among 109 patients exposed to LTG, seven (6.4%) had a sleep disturbance of a severity to required a change in therapy. The descriptions of the sleep disturbance were similar among the patients, and the LTG-induced insomnia appeared to be dose dependent. Unlike the few previous descriptions of LTG-induced insomnia in the literature, no factors predisposing to this adverse effect were identified. CONCLUSIONS: The results of this retrospective review suggest an association between LTG and intolerable insomnia in a small proportion of patients. physicians should inquire about sleep disturbances in patients treated with LTG.
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3/168. Zolpidem tartrate and somnambulism.

    A case is reported in which a patient experienced somnambulistic episodes only after taking zolpidem tartrate for insomnia. Previous to the patient's use of zolpidem tartrate he had never experienced sleepwalking, and once the medication was discontinued the sleepwalking stopped. A search of the literature revealed only two other cases of zolpidem-induced sleepwalking, both involving individuals with a previous history of somnambulism in their youth.
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4/168. Persisting insomnia following traumatic brain injury.

    Persisting insomnia secondary to traumatic brain injury, rarely reported and documented, is described in an adult male following head injury. The neuronal mechanisms underlying this sleep disorder as well as the neuropsychological concomitants and therapeutic approaches are discussed.
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5/168. Helping patients who say they cannot sleep. Practical ways to evaluate and treat insomnia.

    Why do some people spend most of the night tossing and turning while others drop off as quickly as a cat in the sun? There are many possible explanations for problems with falling and staying asleep and, sometimes, patients actually get a lot more sleep than they think they do. Each type of insomnia has its own set of symptoms, which can be used, along with appropriate diagnostic tools, to help in identification. In this article, Dr. Attarian describes differential diagnosis and summarizes the best treatment approaches to the common causes of insomnia.
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6/168. Unexpectedly severe sleep and respiratory pathology in patients with amyotrophic lateral sclerosis.

    Daytime fatigue and sleep disturbance are frequent complaints in patients with amyotrophic lateral sclerosis (ALS). However, polysomnographic data are sparse. Nocturnal respiratory insufficiency may occur despite nearly normal daytime pulmonary function. We describe the clinical presentation and polysomnographic findings in two patients with clinically and electrophysiologically confirmed ALS with minimal weakness but excessive daytime sleepiness. polysomnography in the first patient showed a respiratory disturbance index of 43.5, and profound oxygen desaturations to 62%. The second patient had prolonged periods of hypoventilation, with oxygen saturations oscillating between 86 and 83%. Both patients showed severe sleep maintenance insomnia with a sleep efficiency < 40% and frequent arousals while asleep. Application of continuous positive airway pressure (CPAP) restored normal nocturnal ventilation, blood oxygenation and sleep parameters in the first patient; compliance, however, was poor. The second patient was unable to tolerate CPAP. We conclude that ALS patients with excessive daytime sleepiness or insomnia should undergo polysomnography to adequately diagnose nocturnal respiratory insufficiency and sleep disturbance. compliance with treatment, however, may be poor.
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7/168. "Herbal Ecstasy": a case series of adverse reactions.

    AIMS: To report five cases of adverse effects associated with the ingestion of "Herbal Ecstasy" tablets and discuss possible mechanisms of toxicity. METHOD: Composition of the "Herbal Ecstasy" tablets was determined by discussions with distributors and with MEDSAFE: Reference to relevant texts and an internet and medline literature search was used to identify articles of interest RESULTS: Three patients complained of minor symptoms such as perceptual disturbances, anorexia, inability to sleep, dizziness, palpitations and paresthesia. A fourth patient presented with palpitations and ventricular bigeminy, and a fifth patient presented with headache, vomiting, and a hypertensive crisis. Different brands of "Herbal Ecstasy" have different compositions, some containing caffeine and kava, and one contained ephedrine. CONCLUSION: These five patients presented with adverse reactions to "Herbal Ecstasy" ranging from minor to major. knowledge of the possible mechanisms of toxicity will help plan therapy in cases of major toxicity.
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8/168. Acute abstinence syndrome following abrupt cessation of long-term use of tramadol (Ultram): a case study.

    We report on a patient who had taken the centrally acting analgesic tramadol for over 1 year. The compound had proven to be sufficient to treat her painful episodes related to fibromyalgia. Due to lack of supply while being on a trip, intake of the drug was stopped abruptly, resulting in the development of classical abstinence-like symptoms within 1 week. Abstinence-like symptoms consisted of restlessness and insomnia for which the benzodiazepine lorazepam was given. Diarrhoea and abdominal cramps were treated with the peripherally active opioid loperamide, while bouts of cephalgia were treated with sumatriptan. Diffuse musculoskeletal-related pain and restless leg syndrome (RLS) were treated with dextromethorphan. All these different medications proved to be efficacious as they resulted in the cessation of symptoms. Within 1 week symptoms ceased and the patient regained her normal activities without any sequelae. Although tramadol is considered a non-habit- and non-dependence-forming analgesic, abstinence symptoms are likely to develop following abrupt cessation of intake, especially when the compound had been taken over 1 year. Therefore patients should be advised of such an effect whenever they decide to stop intake or their physician is planning to switch to another medication. To avoid abstinence-like symptoms doses should be slowly tapered down.
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9/168. Withdrawal of synthetic hormones during the perimenopause: a case study.

    A single case study was used to explore the experiences of a perimenopausal woman following withdrawal of synthetic hormones. The respondent, aged 51 years, had experienced severe physiologic, affective and cognitive dysfunction following withdrawal of synthetic hormones. She was approached while attending a family planning clinic in regional new south wales, australia. Information was obtained through a retrospective chart review and in-depth interview. The findings suggest that affective and cognitive dysfunction may be triggered by the sudden withdrawal of artificial hormones in perimenopausal women. Practitioners should be aware that some women, especially those unable to produce sufficient natural hormones, might experience severe physiologic, affective and cognitive dysfunction when hormone supplements are withdrawn. Detailed history-taking and close monitoring following the withdrawal or introduction of hormones may alert practitioners to the incidence of withdrawal symptoms or side-effects. Moving beyond the scientific interpretations, future research should address these concerns and investigate the potential for addiction when hormones are prescribed.
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10/168. Insomnia related to biperiden withdrawal in two schizophrenic patients.

    It is not uncommon for patients who are receiving antipsychotic medication to be given anticholinergic agents, such as biperiden, despite the relative absence of neurological side-effects. Two cases of schizophrenia are reported in which insomnia developed after biperiden withdrawal or reduction. The insomnia continued until biperiden treatment was reinstated, despite the fact that the patients did not exhibit signs or report symptoms indicative of antipsychotic drug-induced neurological side-effects. The occurrence of insomnia following the withdrawal of biperiden or reduction in the dose has not been previously reported. One potential explanation for the insomnia is cholinergic rebound following the withdrawal of biperiden.
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