Signs Of Cerebellar Degeneration Research Articles

Paraneoplastic Cerebellar Degeneration

Paraneoplastic cerebellar degeneration (PCD) presents with acute or subacute onset of ataxia, dysarthria, and intention tremor. In patients older than 50 years, acute or subacute cerebellar degeneration is paraneoplastic in origin in 50% of cases. Paraneoplastic cerebellar degeneration most often precedes a potentially curable remote malignancy. Less often, PCD occurs in a patient with a known malignancy or heralds the onset of a recurrence. The presence of specific antibodies in serum samples helps to guide identification of the occult underlying malignancy. Physicians should entertain the diagnosis of PCD when older patients present with signs of cerebellar degeneration without an obvious cause. A systematic evaluation, including the selection of appropriate imaging and laboratory studies, will often enable physicians to identify the responsible cancer. However, because PCD can precede a cancer by months to years, periodic reevaluation is needed when the cancer remains occult.

Purkinje cell antibodies in a patient with cerebellar disorder: detection of responsible antigenic proteins

A 34-year-old male patient developed a neurological disorder and signs of cerebellar degeneration, with antibodies against Purkinje cells in the serum, a syndrome previously described as "paraneoplastic cerebellar atrophy". Antibody reaction of the patient's serum was demonstrated by immunohistochemistry on sections through the rat and human cerebellum. Purkinje cells demonstrated granular staining of cytoplasmic proteins and proximal dendrites with nuclear sparing. In an immunoblot, the antibodies from the patient's serum reacted with proteins from an extract of rat cerebellum. Only a few distinct proteins from the complex mixture of cerebellar proteins were found to bind with the serum antibodies when using a combination of affinity chromatography and sodium dodecyl sulphate (SDS) gel electrophoresis. The molecular masses of the proteins differed significantly from those identified in patients reported in the literature. Protein denaturation by SDS and 2-mercaptoethanol resulted in a decrease of antibody binding capacity. After immunosuppressive therapy and plasmapheresis, the reaction of the patient's serum with Purkinje cells was greatly diminished; however, only slight clinical improvement was observed. No sign of neoplasm could be found with repeated examinations. The immunological aspects of this case suggest that cerebellar degeneration may be linked to a previously unreported autoimmune response.

The value of computerized tomography in the diagnosis of cerebellar atrophy.

Computerized tomography was performed in 31 patients presenting clinical signs of cerebellar degeneration. CT abnormalities consistent with cerebellar atrophy were found in all cases but one. Specific patterns of abnormality were found in olivo-ponto-cerebellar degeneration and in alcoholic atrophy. The CT findings in spino-cerebellar degeneration were varied, ranging from severe diffuse cerebellar atrophy to normality, possibly according to age and duration of symptoms.

A syndrome of ichthyosis, hepatosplenomegaly and cerebellar degeneration

Two brothers are described with ichthyosis, hepatosplenomegaly and signs of cerebellar degeneration. The ichthyosis was similar to autosomal dominant ichthyosis both clinically and biochemically. No metabolic abnormality has yet been identified to account for this previously undescribed triad of abnormalities.