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1/72. autonomic dysreflexia presenting as a severe headache.

    hypertension, bradycardia, and severe headache have been associated with autonomic dysreflexia. autonomic dysreflexia affects those with spinal transection above the level of T6 after plastic changes of the afferent pathways. This restructuring in the presence of noxious stimuli below the level of the lesion leads to autonomic dysreflexia. The onset of the first episode of autonomic dysreflexia has been documented as soon as 30 days and as late as 13 years after the injury. This report presents a case study of a paraplegic man 8 years after injury with autonomic dysreflexia associated with a urinary tract infection.
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ranking = 1
keywords = injury
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2/72. Sympathetic storms in a child with a midbrain glioma: a variant of diencephalic seizures.

    The authors report the unusual case of a 7-year-old child, one of the youngest reported to date, who developed repeated episodes of sympathetic hyperactivity after surgical resection of a midbrain glioma. These paroxysmal events were similar to previously described diencephalic seizures. However, there was no evidence of epileptogenic activity on electroencephalography, and radiologic imaging did not reveal hydrocephalus or intraparenchymal hemorrhage. In this report, clinical features are described of this patient, along with the novel use of clonidine--a sympathetic blocking agent--in his treatment, published reports are reviewed on diencephalic seizures, and steps are recommended in the treatment of a patient who presents in this manner. The authors believe that diencephalic seizures can present with a spectrum of autonomic features, and treatment should be tailored with the appropriate pharmacologic blockade.
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ranking = 4.0849003125691
keywords = brain
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3/72. Undiagnosed central anticholinergic syndrome may lead to dangerous complications.

    This report describes two cases of central anticholinergic syndrome, the first after general anaesthesia and the other during a prolonged stay in the intensive care unit. The symptoms in both patients resolved soon after physostigmine administration. There was a delay in the diagnosis of central anticholinergic syndrome, which resulted in acute lung injury and unanticipated intensive care unit admission. It is suggested that in cases of abnormal mental recovery after anaesthesia or sedation, the diagnosis of central anticholinergic syndrome should be considered.
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ranking = 0.5
keywords = injury
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4/72. bromocriptine for the management of autonomic dysfunction after severe traumatic brain injury.

    This case report describes a child with severe traumatic brain injury with clinical features of autonomic dysfunction in the immediate post-traumatic period. A history of severe asthma in this child contraindicated the use of beta-blockers, the first line approach, and she was managed with bromocriptine (0.05 mg/kg t.d.s) with good effect.
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ranking = 113.8595412636
keywords = traumatic brain injury, brain injury, traumatic brain, brain, injury, trauma
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5/72. Brainstem-type lewy body disease presenting with progressive autonomic failure and lethargy.

    The authors report an autopsy case characterized by progressive lethargy and autonomic failure with a distinctive pattern of occurrence of lewy bodies. Autonomic dysfunction such as sleep apnea, orthostatic hypotension, dysuria, and hypohidrosis predominated with lethargy, whereas parkinsonism was not apparent. Numerous lewy bodies were widely evident microscopically in brainstem nuclei and the intermediolateral cell columns of the spinal cord, as well as in the sympathetic ganglia, but were rare or absent in the cerebral cortex and other supratentorial structures. Marked neuronal loss was seen in the locus ceruleus, raphe nuclei, dorsal vagal nuclei, and intermediolateral cell columns, but neurons in the substantia nigra, other brain regions, and sympathetic ganglia appeared undiminished. This case represents a specific clinicopathologic form of lewy body disease occurring predominantly in the brainstem, spinal cord, and sympathetic ganglia.
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ranking = 2.4509401875415
keywords = brain
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6/72. Intrathecal baclofen after traumatic brain injury: early treatment using a new technique to prevent spasticity.

    BACKGROUND: In the early course of severe head trauma, the clinical value of intrathecal administration of baclofen to reduce autonomic disorders and spasticity has not been established. methods: We studied four patients (glasgow coma scale score 3 or 4) with autonomic disorders and spasticity who failed to respond to conventional treatment during the early course of head injury. baclofen (25 microg/mL) was infused continuously through an intrathecal catheter inserted at patient bedside and subcutaneously tunneled. When this treatment was successful, the spinal catheter was removed and surgically replaced by another catheter connected to a subcutaneous pump. Clinical follow-up was obtained at 6 months after the head injury. RESULTS: Mean delay for the initiation of intrathecal baclofen was 25 days (range, 21 to 31 days), and optimal dose was 385 /- 185 microg/day. In all patients, the Ashworth score was consistently reduced (3.5 /- 0.5 vs. 4.5 /- 0.5 for upper limbs and 2 /- 0.5 vs. 4.5 /- 0.5 for lower limbs), as were both the frequency and intensity of autonomic disorders. The spinal catheters were used during a mean period of 9.5 /- 1.7 days without complications. All three survivors were equipped with a programmable pump and had a lower Ashworth score at 6 months. Autonomic disorders had disappeared in two patients and remained modest in the remaining patient. CONCLUSION: Continuous administration of baclofen via the intrathecal route using this new technique seems to reduce autonomic disorders and spasticity during the early course of severe traumatic head injury.
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ranking = 230.16384347421
keywords = traumatic brain injury, brain injury, traumatic brain, brain, injury, trauma
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7/72. Decreased parasympathetic activities in Malayan krait (bungarus candidus) envenoming.

    Three patients were bitten by the Malayan krait (bungarus candidus). The patients developed ptosis and generalized muscle weakness which later progressed to respiratory paralysis. All patients showed evidence of decreased parasympathetic activity manifested by mydriasis, hypertension and tachycardia. No specific antivenom was available. All patients received assisted ventilation and supportive treatment. The other forms of treatment included administration of neostigmine, the banded krait (bungarus fasciatus) antivenom (Thai red cross) and plasmapheresis without beneficial response. Two patients recovered. The other patient had permanent brain damage due to anoxia from two episodes of cardiac arrest. While hypertension resolved 6-60 days after admission, mydriasis and tachycardia persisted after discharge in all patients for between 7 days and 2 years. One patient had constipation and defect in micturition which still persisted 2 years after the bite. Decreased parasympathetic activities in Malayan krait bite are perhaps not uncommon and should be examined.
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ranking = 0.81698006251383
keywords = brain
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8/72. Dysautonomia syndrome in the acute recovery phase after traumatic brain injury: relief with intrathecal baclofen therapy.

    In the initial phase of severe head injury, dysautonomic abnormalities are frequent. Within the framework of a prospective study, evaluating the efficacy of continuous intrathecal baclofen therapy (CIBT) on hypertonia during the initial recovery phase of severe head injury, the authors report on the preliminary results of this treatment on paroxysmal dysautonomia about four patients. Continuous intrathecal baclofen infusion was first delivered, for a test period, continuously for 6 days. If a relapse of dysautonomia occurred at the end of the test period, an implantation of a continuous intrathecal infusion pump delivering baclofen was performed. Results were assessed with four continuous variables; duration (days), dose of baclofen per day (microg/d), number of dysautonomic paroxysmal episodes per day, and initial recovery evaluated by a scale of the first initial stages of head injury coma recovery. For three patients: (1) the number of dysautonomic paroxysmal episodes per day and the doses of baclofen during the follow-up period were correlated (p = 0.02, p < 0.001, p = 0.008, respectively, distribution-free test of Spearman), (2) during the test period and the relapse after the test period, the number of paroxysmal episodes and the baclofen dose are correlated to p < 0.05, p = 0.03, p = 0.04, respectively (distribution-free test of Spearman). The second statistical test was used to prove that baclofen doses and number of paroxysmal dysautonomic episodes are correlated independently of the duration of follow-up. The fourth patient improved with CIBT without any recurrence at the end of the treatment test period. For the four patients, recovery score increased during the overall follow-up. In the authors' experience CIBT is very efficient to control paroxysmal dysautonomia during the initial recovery phase in severe head injury, and seems to facilitate recovery.
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ranking = 230.65608007785
keywords = traumatic brain injury, brain injury, traumatic brain, brain, injury, trauma
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9/72. Hereditary neuronal intranuclear inclusion disease with autonomic failure and cerebellar degeneration.

    BACKGROUND: Neuronal intranuclear inclusion disease (NIID), a multiple-system degeneration, occurs usually as a sporadic disorder with onset in childhood. The disease has been found in monozygotic twins and in siblings. In 2 previously described families, the disorder has affected 2 generations. OBJECTIVE: To investigate the clinical, anatomical, and electrophysiological characteristics of NIID that affect the central nervous system and the central and peripheral components of the autonomic nervous system in 2 successive generations of a family. DESIGN: Case report. SETTING: Tertiary care hospital. patients: A 53-year old woman and her sons, aged 28 and 25 years. Symptoms began in childhood in 2 of the 3 cases, and consisted of urinary and fecal incontinence, erectile dysfunction in the men, and recurrent orthostatic hypotension. methods: We used results of clinical neurological evaluations; cranial magnetic resonance imaging; skeletal muscle and sphincter electromyography (EMG); peripheral nerve conduction and bulbocavernosus reflex studies; autonomic function tests; brainstem, visual, somatosensory, and motor evoked potentials; auditory and vestibular testing; metabolic and molecular genetic testing; and muscle and rectal biopsy with immunohistochemistry. RESULTS: We found variable degrees of ocular dysmetria in 2 cases, ataxic dysarthria and limb ataxia in 1, and hyperreflexia in 2. magnetic resonance imaging revealed cerebellar atrophy in all 3 cases and diffuse cerebral cortical atrophy in 1. Results of peripheral nerve conduction studies were normal. Sphincter EMG findings were abnormal in 2 of the 3 cases, and results of autonomic function tests were abnormal in the same 2. The EMG in 1 case revealed a chronic neurogenic pattern in the distal limb muscles. Metabolic and molecular genetic testing revealed no abnormal findings. Results of the muscle biopsy were negative, but results of the rectal biopsy revealed eosinophilic ubiquitinated intranuclear inclusions in neurons. CONCLUSION: Transmission of NIID in 2 generations presenting with autonomic failure and cerebellar ataxia was hereditary.
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ranking = 0.81698006251383
keywords = brain
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10/72. Extensive Riga-Fede disease of the lip and tongue.

    Riga-Fede disease presents in early infancy and is characterized by firm, verrucous plaques arising on the oral mucosal surfaces. These histologically benign lesions occur as a result of repetitive trauma of the oral mucosal surfaces by the teeth. Early recognition of this entity is important, because it may be the presenting sign of an underlying neurologic disorder. We report the case of a 10-month-old boy with extensive Riga-Fede disease involving the lip and tongue that prompted a diagnosis of congenital autonomic dysfunction with universal pain loss.
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ranking = 0.0038816981802973
keywords = trauma
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