perhexiline-maleate and Peripheral-Nervous-System-Diseases

Topics: Electromyography; Humans; Neural Conduction; Perhexiline; Peripheral Nervous System Diseases; Polyneuropathies

Perhexiline maleate is an amphiphilic molecule. Along with many other drugs it is responsible for experimental and, in some instances, clinical lipidoses. Sphingomyelinase deficiency has been evidenced in cell cultures incubated with perhexiline maleate. We describe the occurrence of a similar defect in a patient. The disturbances in the phospholipid turnover which are responsible for the thesaurismosis may originate in the sphingomyelinase deficiency.

Topics: Cerebellar Ataxia; Female; Humans; Lipidoses; Middle Aged; Neuromuscular Diseases; Perhexiline; Peripheral Nerves; Peripheral Nervous System Diseases; Piperidines; Schwann Cells

Two cases of sensory-motor neuropathy attributed to perhexilene maleate are described. The clinical and pathological features of perhexilene neuropathy are discussed. The use of perhexilene should be tempered with caution. Nerve conduction studies may be useful in confirming early neuropathy.

Topics: Angina Pectoris; Female; Humans; Leg; Male; Middle Aged; Neural Conduction; Pain; Paresthesia; Perhexiline; Peripheral Nervous System Diseases

Topics: Adult; Aged; Angina Pectoris; Female; Humans; Liver Cirrhosis; Male; Perhexiline; Peripheral Nervous System Diseases; Piperidines

The use of perhexiline maleate as an antianginal agent is occasionally associated with side effects, particularly neuropathy and liver damage. The reason why some individuals develop these toxic reactions is not clear, though some evidence suggests that they may result from impaired oxidative metabolism, due to genetic or hepatic factors, and consequential accumulation of the drug in toxic concentrations. Drug oxidation was measured with an oxidation phenotyping procedure in 34 patients treated with perhexiline, 20 of whom had developed neuropathy and 14 of whom had not. Most of the 20 patients with neuropathy, but not the unaffected patients, showed an impaired ability to effect metabolic drug oxidation. This impairment was independent of hepatic function, concurrent drug therapy, or tobacco or alcohol consumption. The fact that the ability to oxidise several drugs is genetically controlled points to a genetic susceptibility to developing neuropathy in response to perhexiline. Routine determination of the drug oxidation phenotype might lead to safer use of perhexiline by predicting patients who may be more at risk of developing a neuropathic reaction associated with its long-term use.

Topics: Adult; Aged; Alcohol Drinking; Angina Pectoris; Debrisoquin; Female; Humans; Isoquinolines; Liver Function Tests; Male; Middle Aged; Oxidation-Reduction; Perhexiline; Peripheral Nervous System Diseases; Piperidines; Smoking

In a 78-year-old woman receiving perhexiline maleate for intractable angina pectoris, a syndrome of parkinsonism and peripheral neuropathy developed. The neuropathy was confirmed by electromyographic and nerve conduction studies. The parkinsonism and peripheral neuropathy disappeared when perhexiline maleate was discontinued.

Topics: Aged; Angina Pectoris; Female; Humans; Parkinson Disease, Secondary; Perhexiline; Peripheral Nervous System Diseases; Piperidines

Topics: Cells, Cultured; Fibroblasts; Humans; Lipidoses; Perhexiline; Peripheral Nerves; Peripheral Nervous System Diseases; Piperidines

Topics: Aged; Electromyography; Humans; Neural Conduction; Paresthesia; Perhexiline; Peripheral Nervous System Diseases; Vasodilator Agents