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Biomedical subjects

D J Dickoff

Publications and source records attributed to D J Dickoff.

6 recordsLinked to original sources

Erroneous diagnosis corrected after 28 years. Not spinal muscular atrophy with ophthalmoplegia but minicore myopathy.

OBJECTIVE: To correct, after 28 years, the previously reported diagnosis of ophthalmoplegia in a patient with presumed childhood spinal muscular atrophy. DESIGN: Clinical follow-up, laboratory, electrophysiologic, and muscle biopsy data are provided. RESULTS: The findings of clinical follow-up examination, electrophysiologic tests, and histologic examination of muscle specimens led to a revised diagnosis of minicore myopathy. CONCLUSIONS: Spinal muscular atrophy was diagnosed in 1967, before histochemical techniques for examining muscle tissue and quantitative electromyography became widely available. Modern laboratory techniques later made the diagnosis of minicore myopathy possible. Progressive external ophthalmoplegia has been described in 24% of patients with minicore myopathy, but there have been only 7 reports of ophthalmoplegia with spinal muscular atrophy since 1954, and some of these diagnoses have been questioned.

Adult↗

HTLV-1 in acquired adult myopathy.

We report a 53-year-old Jamaican man with 20 years of progressing weakness involving proximal limb muscles and neck flexors. Serum CK was 1100 IU/L. EMG demonstrated spontaneous activity, myopathic motor units, and full recruitment patterns in weak muscles. Muscle biopsy revealed marked myofiber degeneration with extensive fibrosis, suggesting a chronic myopathic process. HTLV-1 antibody was present in serum in high titers by ELISA and Western blot. Immunohistochemistry with rabbit polyclonal antisera to HTLV-1 showed rare staining myocytes. PCR demonstrated HTLV-1 DNA in frozen muscle tissue. This myopathy, associated with HTLV-1 infection, has clinical and pathological features similar to a dystrophy. We recommend serological screening for HTLV-1 in cryptogenic adult myopathies.

HTLV-I Infections↗

Striatal syndrome following hyponatremia and its rapid correction. A manifestation of extrapontine myelinolysis confirmed by magnetic resonance imaging.

Several pathologic reports have indicated the occurrence of extrapontine sites of myelinosis associated with electrolyte disturbance and its rapid correction. Clinical evidence of the presence of such lesions, however, have rarely been described. We report a case of a 50-year-old man who following rapid correction of electrolyte imbalance developed a profound striatal syndrome. The clinical manifestations, the course of his illness as well as response to therapy, confirmation of lesion sites by magnetic resonance imaging (MRI), and abnormalities on visually evoked potentials are described in detail.

Basal Ganglia Diseases↗

Delayed neurotoxicity after ingestion of carbamate pesticide.

We studied a patient who ingested 27 gm (500 mg/kg) of carbaryl (1-naphthyl N-methylcarbamate), a popular carbamate pesticide. After he recovered from acute cholinergic toxicity, acute weakness of arms and legs was accompanied by electrophysiologic findings consistent with axonal peripheral neuropathy. Recovery began at 1 week and continued for 9 months. A similar delayed neuropathy has been described with organophosphates but not with carbamate insecticides.

Adult↗