Selective loss of Purkinje cells in a patient with anti-glutamic acid decarboxylase antibody-associated cerebellar ataxia
Open Access
- 1 February 2007
- journal article
- case report
- Published by BMJ in Journal of Neurology, Neurosurgery & Psychiatry
- Vol. 78 (2) , 190-192
- https://doi.org/10.1136/jnnp.2006.091116
Abstract
Anti-glutamic acid decarboxylase antibody is associated with the development of progressive cerebellar ataxia and slowly progressive insulin-dependent diabetes mellitus. Previously, the neurophysiological characteristics of IgG in the cerebrospinal fluid of a patient with anti-glutamic acid decarboxylase antibody-associated progressive cerebellar ataxia and slowly progressive insulin-dependent diabetes mellitus were reported. Using a voltage-gated whole-cell recording technique, it was observed that the IgG in the cerebrospinal fluid of the patient selectively suppressed the inhibitory postsynaptic currents in the Purkinje cells. The patient died from aspiration pneumonia. Postmortem examination showed almost complete depletion of the Purkinje cells with Bergmann gliosis. Therefore, the main cause of cerebellar ataxia observed in this case may be attributed to the near-complete depletion of the Purkinje cells. In this paper, the pathomechanisms underlying Purkinje cell damage are discussed.Keywords
This publication has 16 references indexed in Scilit:
- Dual impairment of GABAA- and GABAB-receptor-mediated synaptic responses by autoantibodies to glutamic acid decarboxylasePublished by Elsevier ,2003
- Mechanisms underlying cerebellar motor deficits due to mGluR1‐autoantibodiesAnnals of Neurology, 2002
- Glutamic acid decarboxylase autoantibodies and neurological disordersNeurological Sciences, 2002
- Niemann–Pick type C disease: Accelerated neurofibrillary tangle formation and amyloid β deposition associated with apolipoprotein E ε4 homozygosityAnnals of Neurology, 2002
- Pathological evidence of encephalomyelitis in the stiff man syndrome with anti-GAD antibodiesJournal of Clinical Neuroscience, 2002
- Stiff person syndromeNeurology, 2001
- Cerebellar Ataxia With Anti–Glutamic Acid Decarboxylase AntibodiesArchives of Neurology, 2001
- Inflammatory infiltrates and complete absence of Purkinje cells in anti-Yo-associated paraneoplastic cerebellar degenerationActa Neuropathologica, 1996
- Prevalence of autoantibodies to the 65- and 67-kD isoforms of glutamate decarboxylase in insulin-dependent diabetes mellitus.Journal of Clinical Investigation, 1993
- Relationships among residual β cells, exocrine pancreas, and islet cell antibodies in insulin-dependent diabetes mellitusMetabolism, 1993