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Hematological malignancies include several diseases that may affect the peripheral nervous system (PNS) through various mechanisms. A common and challenging situation is represented by the occurrence of an active peripheral neuropathy in a patient with a supposed inactive hematological disorder.We report clinical, electrophysiological, biological, and pathological data of 8 patients with latent malignant hemopathies (most were considered in remission): B-cell chronic lymphocytic leukemia in 3 patients, B-cell lymphoma in 1 patient, low-grade non-Hodgkin's lymphoma in 1 patient, Waldenström's macroglobulinemia in 1 patient, smoldering multiple myeloma in 1 patient, and monoclonal gammopathy of undetermined significance in 1 patient.In all these cases, the nerve biopsy (NB) helped to diagnose the hematological relapse or detect a pathological mechanism linked to the hematological disorder: epineurial lymphocytic infiltration in 5 patients (including one with antimyelin-associated glycoprotein antibodies), cryoglobulin deposits in 1 patient, chronic inflammatory demyelinating polyneuropathy in 1 patient, and necrotizing vasculitis in 1 patient. In each case, pathological findings were crucial to select the adequate treatment, leading to an improvement in the neurological and biological manifestations.These observations illustrate the value of NB and the need for active collaboration between neurologists and hematologists in such cases.

Purpose: Somatosensory evoked potentials (SEPs) offer complementary results to those of nerve conduction studies and contribute to the electrodiagnostic criteria of chronic inflammatory demyelinating polyradiculoneuropathy., Methods: We performed nerve conduction studies and SEPs in patients with symmetrical motor weakness, areflexia, and/or sensory disturbances lasting for at least 8 weeks. We determined two groups according to the electrodiagnostic criteria of the European Federation of Neurological Societies. Group 1 included patients who met the definite or probable electrodiagnostic criteria, and group 2 included patients who met the possible electrodiagnostic criteria. We also compared SEPs results with those of controls (group of healthy subjects)., Results: Sixteen patients (14 men; mean age, 59 ± 17.3 years) were included in the study. The latencies of potentials N9, N13, N7, and N22 and the intervals N9-N13 and N7-N22 were significantly increased in patients compared with controls. The N9/iP14 amplitude ratio was significantly lower in patients. There was no significant difference in the latencies of SEPs between the two groups of patients., Conclusions: We confirm the contribution of SEPs as complementary information to nerve conduction studies in chronic inflammatory demyelinating polyradiculoneuropathy diagnosis. In addition to the usual abnormalities, a decrease in the N9/iP14 amplitude ratio could potentially be used as an electrodiagnostic criterion.

Background: Amyotrophic lateral sclerosis (ALS) is the most serious form of degenerative motor neuron disease in adults, characterized by upper and lower motor neuron degeneration, skeletal muscle atrophy, paralysis, and death. High prevalence of malnutrition and weight loss adversely affect quality of life. Moreover, two thirds of patients develop a hypermetabolism of unknown cause, leading to increased resting energy expenditure. Inasmuch as lipids are the major source of energy for muscles, we determined the status of lipids in a population of patients with ALS and investigated whether lipid contents may have an impact on disease progression and survival., Methods: Blood concentrations of triglycerides, cholesterol, low-density lipoprotein (LDL), and high-density lipoprotein (HDL) were measured in a cohort of 369 patients with ALS and compared to a control group of 286 healthy subjects. Postmortem histologic examination was performed on liver specimens from 59 other patients with ALS and 16 patients with Parkinson disease (PD)., Results: The frequency of hyperlipidemia, as revealed by increased plasma levels of total cholesterol or LDL, was twofold higher in patients with ALS than in control subjects. As a result, steatosis of the liver was more pronounced in patients with ALS than in patients with PD. Correlation studies demonstrated that bearing an abnormally elevated LDL/HDL ratio significantly increased survival by more than 12 months., Conclusions: Hyperlipidemia is a significant prognostic factor for survival of patients with amyotrophic lateral sclerosis. This finding highlights the importance of nutritional intervention strategies on disease progression and claims our attention when treating these patients with lipid-lowering drugs.

Spinal muscular atrophy (SMA) is caused by SMN1 gene deletions or mutations, and ALS is the most frequent motor neuron condition in adults. The authors describe three families in which ALS and SMA coexist. The authors found that no SOD1 mutation was found within these families; all three ALS cases had at least two SMN1 copies; and an abnormal SMN1 gene locus did not explain the co-occurrence of these two motor neuron disorders in these families.