Spinal cord herniation: a missing piece in the pathogenesis of amyotrophic lateral sclerosis and multiple sclerosis?

Background: Idiopathic spinal cord herniation (SCH) is a rare and often misdiagnosed condition characterized by displacement of the spinal cord through an anterior defect of the dural sac. This condition determines continuous focal trauma of the spinal cord, causing slowly progressive myelopathy. The peculiar MR scan findings, particularly sagittal T(2)-weighted images, allow its recognition.

Neurosarcoidosis. Clinical description of 7 cases with a proposal for a new diagnostic strategy.

Objective: Chronic involvement of the nervous system is relatively rare in sarcoidosis. We describe 7 cases that fulfil Zajicek's criteria for neurosarcoidosis (NS) and propose some modifications to such criteria.

Materials And Methods: The patients were admitted for various neurological syndromes: 2 cases presented with chronic lymphocytic meningitis, 4 with spinal cord symptoms, one case was initially confused with multiple sclerosis.

Anti-gabaergic neuron autoantibodies in a patient with stiff-man syndrome and ataxia.

We describe a patient with autoimmune insulin-dependent diabetes whose original symptoms of trunk stiffness and rigidity of the abdomen were followed three years later by a pancerebellar syndrome. An autoantibody (autoAb) against GABAergic neurons was found in the patient's serum and cerebrospinal fluid (CSF); on Western blot, this autoAb recognized a 64-kDa antigen of cerebellar protein. The detection of this antibody in a case with ataxia suggests that a spectrum of different neurological diseases may be observed in patients harbouring anti-GABAergic neuron autoAb and supports the concept that factors other than autoAb contribute to the clinical presentation of these disorders.

Circulating autoantibodies against central nervous system (CNS) antigens in neurological diseases.

A number of investigators have reported the detection of circulating autoantibodies directed against serum and cerebrospinal fluid (CSF) neuronal antigens in certain neurological clinical conditions. Using an immunohistochemical technique, we examined the sera and (when available) the CSF from 120 patients with several neurological disorders and 40 controls in order to analyze the incidence and specificity of the detection of these autoantibodies. Circulating autoantibodies were found in 3 patients with cerebellar degeneration and in 3 patients with stiff-man syndrome, and different staining patterns were revealed in the same disease.