ABSTRACT
A patient with anti-Hu antibodies, small-cell lung carcinoma, and autopsy-proven subacute sensory neuropathy had early slowing of motor and sensory conduction velocities. In the peripheral nerves, chronic demyelinating and remyelinating lesions with axonal degeneration were associated with an inflammatory reaction consisting of CD8+ T cells and CD68+ macrophages. On immunohistochemical testing, the patient's serum did not react with normal nerve, suggesting that the Hu proteins were not the target of the inflammatory reaction in the nerve.
Subject(s)
Demyelinating Diseases/pathology , Ganglia, Sensory/pathology , Nerve Tissue Proteins , Paraneoplastic Syndromes/immunology , Paraneoplastic Syndromes/pathology , RNA-Binding Proteins/immunology , Aged , Autoantibodies/blood , Carcinoma, Small Cell/complications , Demyelinating Diseases/immunology , ELAV Proteins , Fatal Outcome , Ganglia, Sensory/immunology , Humans , Immunoenzyme Techniques , Lumbosacral Plexus/immunology , Lumbosacral Plexus/pathology , Lumbosacral Plexus/ultrastructure , Lung Neoplasms/complications , Male , Microtomy , Peroneal Nerve/immunology , Peroneal Nerve/pathology , Peroneal Nerve/ultrastructure , Spinal Nerve Roots/immunology , Spinal Nerve Roots/pathologySubject(s)
Arm/innervation , Sensation Disorders/physiopathology , Chronic Disease , Disease Progression , Humans , Male , Middle AgedABSTRACT
The association of chronic inflammatory demyelinating polyneuropathy (CIDP) and carcinoma has rarely been reported and its relevance is debated. Thirty three consecutive patients with probable or definite CIDP (idiopathic or associated with M protein) were investigated. Three patients with definite CIDP had a concomitant carcinoma. One had an IgM paraprotein. Steroids and intravenous immunoglobulins were effective.
Subject(s)
Carcinoma/complications , Demyelinating Diseases/complications , Nervous System Neoplasms/complications , Aged , Carcinoma/physiopathology , Chronic Disease , Demyelinating Diseases/physiopathology , Humans , Inflammation , Male , Middle Aged , Nervous System Neoplasms/physiopathology , Neural Conduction/physiologyABSTRACT
Particular clinical pictures of ALS may occur during the course of some "benign gammapathies". We observed 12 patients (age range 57 to 74 years; 9 men-3 women) with initially benign gammapathy (9 IgG, 1 IgA, 2 IgM) associated to a clinical picture of progressive anterior horn and pyramidal tract involvement. These cases led us to recognize some particularities of gammapathy-associated ALS: Relative frequency of asymmetrical clinical manifestations, rarity of bulbar signs; Decrease of sensory nerve conduction velocities without evidence of sensory clinical symptoms (8 out 9); Increase CSF protein content including the monoclonal component, axonal degeneration and immunostaining evidence of the paraprotein fixation observed on nerve biopsies (5 out 7 cases). Association of ALS and gammapathy is not fortuitous as shown by epidemiology, experiments, pathology and effects of different immunological treatments as related in this study. Demonstration of infra-clinical neuropathy face to a clinical syndrome of ALS should prompt too careful screening for a gammapathy.
