Cyanide-induced parkinsonism: a clinicopathologic report.

An 18-year-old man ingested 975 to 1,300 mg of potassium cyanide in a suicide attempt. He was treated and survived the poisoning episode, but then had severe parkinsonian syndrome, characterized primarily by akinesia and rigidity. He died 19 months after the drug overdose. At autopsy, major destructive changes were found in the globus pallidus and putamen, whereas the melanin-containing zone of substantia nigra was intact. This is the first clinicopathologic report of parkinsonism as a result of cyanide poisoning.

Chronic pain syndromes and their treatment. I. Cluster headache.

Cluster headache is a form of unilateral headache which, in the past, has been very resistant to treatment. Lithium carbonate has been shown to be the only reliably effective treatment in this condition and, although only a few cases have so far been reported in the literature they have, with very few exceptions, been successful. Therefore we selected five patients who at one time or another had presented with cluster headache and who had no relief from conventional treatment, and treated them with lithium carbonate. This has been highly successful. Some patients who had suffered from the disease for many years have been completely or almost completely relieved of their affliction, while others have been markedly improved to the point where they can now function normally, both socially and professionally. The possible modes of action of lithium in this condition are discussed.

Lumbo-sacral radiculopathy induced by radiation.

Two patients had lumbo-sacral radiculopathy following radiation treatment of cancer. Twenty previously reported cases were similar. The clinical picture is one of progressive motor and sensory loss in the legs, usually appearing within a year after radiation, but sometimes delayed up to several years. Experimental studies quoted indicate greater vulnerability of peripheral nerves to ionizing radiation than has been previously recognized. Lumbo-sacral radiculopathy is readily produced in the experimental animal (rat) and affords an experimental model closely resembling the human cases reported.

Mucolipidosis III (pseudo-Hurler polydystrophy): Clinical and laboratory studies in a series of 12 patients.

Mucolipidosis III (pseudo-Hurler polydystrophy) is an autosomal recessively inherited Hurler-like disorder without mucopolysacchariduria. Previous reports have noted a constellation of laboratory features similar to that described for mucolipidosis II (I-cell disease). Studies were carried out on a series of 15 patients. Twelve were found to have changes in serum and cultured fibroblasts which consisted of marked elevations of several acid hydrolases in serum with low levels of the same enzymes in cultured cells, a marked increase in dense cytoplasmic inclusions and abnormal radioactive sulfate kinetics. The clinical features of these 12 patients comprise a phenotypic entity. Despite clinical similarity, the 3 remaining patients were not felt to represent mucolipidosis III. The basic defect in mucolipidosis III remains unknown, but is suggested that the defect is similar to that of mucolipidosis II, from which it must be distinguished clinically.

Bilateral carpal tunnel syndrome in childhood. A report of two sisters with mucolipidosis III (pseudo-Hurler polydystrophy).

Bilateral carpal tunnel syndrome is described in two sisters aged six and seven years, complicating the course of mucolipidosis III (pseudo-Hurler polydystrophy). The literature regarding carpal tunnel syndrome in childhood is reviewed with particular reference to diagnostic features and the association, in bilateral cases, with the mucopolysaccharidoses and mucolipidoses.

The nature of essential tremor.

The literature concerning essential tremor is confusing. Some accounts describe tremor of a resting type and its accompaniment by other neurological abnormalities. Critical analysis of the pertinent literature leaves some question as to the validity of these observations.Clinical analysis of 34 patients with essential tremor, personally observed during a four-year period, reveals this to be a monosymptomatic condition. The tremor was usually of both a postural and action type and resting tremor was not observed. There were no other neurological abnormalities. Serious disability was infrequent and, when present, was related to upper limb tremor. Essential tremor should be readily distinguishable from other central nervous system diseases but it may be confused with Parkinson's disease or cerebellar ataxia.

Chorea complicating polycythemia rubra vera.

A patient with polycythemia rubra vera and chorea is described and the literature on this rare combination is reviewed. This is the first report of the association of chorea and polycythemia in a male patient.The contrasting features of other forms of diffuse cerebral pathology which may be accompanied by chorea, namely disseminated lupus erythematosus, Henoch-Schönlein purpura and rheumatic chorea, are discussed.