Faculty Bibliography

Diabetes mellitus leads to several recognizable clinicopathologic neuropathic syndromes. Diagnosis and evaluation requires a thorough history and neurologic examination, electrophysiologic studies, blood studies and, in selected cases, cerebrospinal fluid analysis and nerve and muscle biopsy. Microangiopathy is the leading cause of diabetic neuropathy associated with metabolic, vascular ischemic and immunologic injury. Tight glycemic control and symptomatic therapy is beneficial in a minority of patients but does not prevent the relentless progression of symptoms and signs. Intravenous immune globulin is a novel therapy in patients with mononeuropathy multiplex, primary demyelinating neuropathy and peripheral nerve T-cells microvasculitis associated with C5b-9 membrane attack complex protein deposits

The series on treatable neuromuscular disorders continues with the present article on the peripheral nervous system manifestations of Lyme neuroborreliosis

BACKGROUND: The cause of amyotrophic lateral sclerosis (ALS) is not known, and there is no effective treatment. Cell death may be caused by oxidative damage. Selegiline hydrochloride (Eldepryl) is a monoamine oxidase-B inhibitor with antioxidant properties. OBJECTIVE: To determine if selegiline affects the clinical course of patients with ALS. DESIGN: Six-month, double-blind, placebo-controlled study of 133 patients with classical ALS and symptoms for less than 3 years. The primary end point to indicate effectiveness was the rate of change of the Appel ALS total score, an index of disease severity that incorporates strength and function in limbs, respiratory function, and bulbar function. RESULTS: Of the 133 patients, 67 were randomized to receive selegiline and 66 to receive placebo. One hundred four patients (53 in the selegiline group and 51 in the placebo group) completed the 6-month trial. Both groups were comparable for baseline characteristics and mean Appel ALS total score (70.5 points for the selegiline group and 70.6 for the placebo group). There was no difference in the rate of progression as measured by the Appel ALS total score, showing an average increase of 22 points in 6 months. The monthly rate of change was 3.4 for the selegiline group and 3.5 for the placebo group. There was 1 adverse reaction: worsening depression. Seven patients died during the study (4 in the selegiline group and 3 in the placebo group). CONCLUSION: Selegiline treatment had no significant effect on the rate of clinical progression or outcome of ALS

Diabetes mellitus leads to several recognizable clinicopathologic neuropathic syndromes. Diagnosis and evaluation requires a thorough history and neurologic examination, nerve conductions and needle electromyography (EMG), blood studies, consideration of cerebrospinal fluid analysis, and nerve and muscle biopsy in the most severely affected patients. Microangiopathy is the commonest cause of diabetic neuropathy, associated with potentially reversible metabolic, immunologic, or ischemic injury. Tight glycemic control and symptomatic therapy is beneficial in some patients but does not prevent progression of neuropathy especially in patients with severe motor and gait disability. Intravenous immune globulin is a novel therapy in diabetic patients. It may be considered in selected patients well characterized by clinical, electrophysiologic, histopathologic studies, and one of the following progressive syndromes: mononeuropathy multiplex, primary demyelinating motor or sensorimotor neuropathy, and peripheral nerve perivasculitis or microvasculitis associated with vascular membrane attack complex protein deposits

This article is an introduction to the historical background, clinical and laboratory diagnosis, pathogenesis, and treatment of vasculitis involving the peripheral and central nervous system. It also provides a background for the articles that follow

Central nervous system (CNS) vasculitis refers to primary and secondary disorders of the CNS vasculature. Most authorities agree that CNS vasculitis is a potentially serious disorder; therefore, prompt diagnosis and initiation of therapy are high priorities in treatment. Remarkable progress has been made in the diagnosis, evaluation, and treatment of this disorder. This article examines many aspects of the radiographic evaluation of CNS vasculitis