Enayat Niakan

Diabetic autonomic neuropathy

The incidence of autonomic dysfunction as a complication of diabetes mellitus is reported to be as high as 20% to 40%. Symptoms of diabetic autonomic neuropathy (DAN) are often vague, and signs difficult to detect on routine physical examination. The early diagnosis of DAN is possible by utilizing several simple noninvasive tests, which may also be helpful in localizing the lesion(s) to specific autonomic pathways. DAN may affect multiple organ systems, to include cardiovascular, gastrointestinal, genitourinary and/or neuroendocrine, and may, in fact, be life-threatening. The same metabolic disturbances of somatic peripheral nerve may also be responsible for DAN. Like somatosensory neuropathy, definitive therapy for DAN is not yet satisfactory, although multiple chemotherapeutic agents have been tried and warrant further investigation.

The clinical spectrum of inflammatory-angiopathic neuropathy.

Thirty-three patients with inflammatory-angiopathic neuropathy diagnosed by sural nerve biopsy, were investigated to determine the underlying disease. Twenty-six patients had symmetrical sensorimotor polyneuropathy and seven had mononeuropathy multiplex. An aetiology for inflammatory-angiopathic neuropathy was found in only eight patients: typical collagen vascular disease in five and malignant tumour in three. Sixteen patients received prednisone and/or immunosuppressive drug therapy and 12 (75%) responded to treatment. This study demonstrates that typical collagen vascular diseases are not the most common cause of inflammatory-angiopathic neuropathy, that symmetrical polyneuropathy is seen in 75.8% of inflammatory-angiopathic neuropathy patients. Prednisone and/or immunosuppressive agents appear effective regardless of aetiology.

Tubular aggregates: their association with myalgia.

Three thousand consecutive muscle biopsies were reviewed for the presence of tubular aggregates and their association with clinical symptomatology. Tubular aggregates were detected in 19 patients (0.6%). Twelve of these nineteen patients had severe myalgia, and the most abundant tubular aggregates were found in biopsies of patients with myalgia. Seven patients had only myalgia as their clinical symptomatology with normal physical examination. An additional five patients with tubular aggregates and myalgia had concomitant amyotrophic lateral sclerosis (2) or neuropathy (3). The high incidence of myalgia associated with tubular aggregates in our patients and the fact that tubular aggregates originate from sarcoplasmic reticulum suggest a role played by this structure in the pathogenesis of myalgia.

Childhood dermatomyositis in monozygotic twins

Identical twins who developed childhood dermatomyositis 2 weeks apart following upper respiratory infections are reported. Observations suggest that genetic influence on host susceptibility may play a role in childhood dermatomyositis and that a combination of host and environmental factors is most likely necessary to produce the disease.

Hydrazine toxicity, pyridoxine therapy, and peripheral neuropathy.

Excerpt To the editor: A patient who had hydrazine intoxication was successfully treated with megadoses of pyridoxine hydrochloride (vitamin B6) and subsequently developed a sensory polyneuropathy ...