ABSTRACT
Lambert-Eaton myasthenic syndrome (LEMS) is an immune-mediated disorder of the neuromuscular junction that rarely is associated with cerebellar ataxia (CA). We describe two patients with non-paraneoplastic LEMS associated with CA who showed high levels of anti-P/Q-type voltage-gated calcium channels antibodies in the serum and cerebrospinal fluid, and reduced CMAP with increment after brief maximum voluntary contraction in electrophysiological studies. We suggest that LEMS should be considered in the differential diagnosis of patients with CA.
Subject(s)
Cerebellar Ataxia/complications , Lambert-Eaton Myasthenic Syndrome/complications , Action Potentials/physiology , Adult , Antibodies/blood , Calcium Channels/immunology , Cerebellar Ataxia/blood , Cerebellar Ataxia/cerebrospinal fluid , Cerebellar Ataxia/drug therapy , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Lambert-Eaton Myasthenic Syndrome/blood , Lambert-Eaton Myasthenic Syndrome/cerebrospinal fluid , Lambert-Eaton Myasthenic Syndrome/drug therapy , Male , Middle Aged , Muscle, Skeletal/physiopathology , Neural Conduction/physiology , Paraneoplastic Syndromes/blood , Paraneoplastic Syndromes/complicationsSubject(s)
Antibody Formation/immunology , B-Lymphocytes/immunology , Cerebrospinal Fluid/cytology , Paraneoplastic Syndromes, Nervous System/immunology , T-Lymphocytes/immunology , Adult , Antibodies, Neoplasm/cerebrospinal fluid , Antibodies, Neoplasm/immunology , Antibodies, Neoplasm/physiology , Antigens, Neoplasm/analysis , B-Lymphocytes/physiology , Cerebrospinal Fluid/immunology , Child , Humans , Lambert-Eaton Myasthenic Syndrome/cerebrospinal fluid , Lambert-Eaton Myasthenic Syndrome/immunology , Lambert-Eaton Myasthenic Syndrome/physiopathology , Neuroblastoma/cerebrospinal fluid , Neuroblastoma/immunology , Paraneoplastic Syndromes, Nervous System/cerebrospinal fluid , Receptors, Antigen, T-Cell, gamma-delta/immunology , T-Lymphocyte Subsets/immunology , T-Lymphocytes/physiologyABSTRACT
In two men presenting with muscle weakness and disturbances of equilibrium neurophysiological examination by repeated stimulations revealed responses suggestive of Lambert-Eaton syndrome. In the first month of the disease very high levels of anti-Hu antibody were found in the serum and CSF, betraying a malignant lesion. This was confirmed by autopsy 4 months later in one patient and by bronchial biopsy 16 months later in the other patient. Both had small-cell lung carcinoma associated with paraneoplastic encephalomyelitis.
