Thiamine-responsive acute neurological disorders in nonalcoholic patients.

Wernicke's encephalopathy (WE) is most commonly associated with alcoholism, although other causes have also been implicated. In the years 1994-1997, 9 patients with no history of alcohol abuse presented with acute signs of ophthalmoplegia or nystagmus and ataxia which resolved within 48 h after intravenous thiamine. There were 7 women and 2 men aged 17-57 (7 below the age of 30). Precipitating events included vomiting 2, drastic weight-reducing diet 2, renal colic in a postpartum woman 1, colonic surgery 2 and chronic hemodialysis 1. In 2 patients there was no obvious precipitating event but their history was suggestive of a genetic predisposition. Mental changes were slight or absent in all patients and all of them made good functional recovery. These cases suggest that the diagnosis of WE should be considered more often in nonalcoholics in various clinical settings.

Cefuroxime-induced encephalopathy.

We describe four patients who developed an encephalopathic syndrome characterized by obtundation or stupor, myoclonic jerks, and asterixis in association with cefuroxime therapy. Three patients had renal failure. These cases suggest that cefuroxime in overdose or in conventional doses in patients with renal failure can cause a reversible encephalopathy. This syndrome may have been unrecognized because it usually occurs in severely ill patients with additional causes for encephalopathy.

Spontaneous vertical eye movements associated with pontine lesions.

Ocular bobbing, dipping and "reversed" ocular dipping were observed and recorded in two patients presenting a severe neurological symptomatology: quadruplegia and coma in one and locked-in syndrome in the other. CT scan showed a pontine infarction in both these patients. This is the first time that the ocular dipping and "reversed" ocular dipping are related to such an anatomic structure.

Motor neuron disease in textile factory workers.

Five cases of motor neuron disease occurred during the period 1983-1987 in 4 small and medium-sized textile factories. These cases were 45% of 11 motor neuron disease patients diagnosed in this period in a population of about 300,000 persons. This observations may be relevant to toxic occupational hazards in the pathogenesis of motor neuron disease.

Peripheral neuropathy and cerebellar syndrome associated with amiodarone therapy.

A 52-year-old woman developed symmetric sensorimotor polyneuropathy and cerebellar syndrome after 24 months of amiodarone treatment. Sural nerve biopsy revealed mild demyelination and many inclusion bodies in the axoplasm and in Schwann cell cytoplasm. Improvement in her condition was evident 6 months after discontinuing amiodarone.

Myophosphorylase deficiency: the course of an unusual congenital myopathy.

A 59-year-old man had proximal weakness and wasting that started in early childhood. EMG was "myopathic," serum CK activity was increased, and muscle biopsy showed accumulations of glycogen. Biochemical studies revealed elevated glycogen concentration and absence of myophosphorylase activity. This unusual presentation of a long-standing, painless, and quite static weakness due to myophosphorylase deficiency represents another example of clinical heterogeneity.

Vitamin B12 deficiency neuropathy: sural nerve biopsy study.

A 40-year-old woman developed neuropathy and myelopathy with vitamin B12 deficiency without anemia. Motor and sensory nerve conduction velocities were moderately decreased. Sural nerve biopsy showed the coexistence of demyelination and axonal degeneration.

Primary amyloidosis with peripheral neuropathy and signs of motor neuron disease.

A 51-year-old man had primary amyloidosis, with typical amyloid neuropathy and signs of motor neuron disease, including widespread fasciculation in limb muscles, tongue atrophy and fasciculation, swallowing and chewing difficulty, symmetric hyperreflexia, and bilateral Hoffmann's signs. Fasciculations, fibrillations, and positive sharp waves were found in electromyography of all muscles tested. Motor nerve conduction velocities were moderately slow. Lambda chains were detected in serum and CSF. Amyloid was found in sural nerve biopsy. This combination of amyloid neuropathy and features of amyotrophic lateral sclerosis is related to the motor neuron disease of plasma cell dyscrasias.

The use of the portal system for the transplantation of a neonate kidney graft in a child with Wilms' tumor.

A single an-encephalus neonate kidney graft was transplanted into the portal system of a 6-year-old recipient who had previously undergone removal of the right kidney and inferior vena cava because of Wilms tumor. The left kidney ceased to function shortly thereafter. The child was supported very poorly on hemodialysis, and showed repeated very high levels of cytotoxic antibodies in her serum. The first cross-negative kidney graft that was available harbored two main arteries and duplicate collecting system with two very thin ureters. These vascular anatomic and pathologic variations of both donor graft and recipient necessitated the use of the portal system for renal graft venous drainage and the aorta for the graft revascularization. The ureters that had pinpoint-like lumen were inserted together into the lumen of the native ureter stump and fixated. One year after the transplantation the serum creatinine level is 1.8 mg/dL.

Mu and alpha rhythm in comatose children.

Three anoxic comatose children had EEG alpha-like activity and in two of them mu rhythm was recorded. The paradoxical appearance of these electrical activities in comatose children seems to indicate a grave prognosis. A possible role for barbiturate treatment in this phenomenon is not excluded.