What is Subacute Cerebellar Degeneration?

Subacute Cerebellar Degeneration
Spatial characteristics of cerebellar cortical degeneration in the β-III spectrin deficient beagle after calbindin-immunohistochemistry and haematoxylin counterstain.(A) Navigator figure depicting the sampled areas. (B) The ventral aspects of the vermis show least numeric loss of calbindin-positive (brown) Purkinje cells (PC), a narrow subarachnoid space (SAS) and remnants of the external germinative cell layer (EGL). Furthermore, the granule layer (GL) is well populated and its histoarchitecture is preserved. Degenerative changes are restricted to dystrophic dendrites (white arrowhead). Purkinje cell loss becomes increasingly evident in the dorsal vermis (C) and the ansiforme lobulus of the cerebellar hemispheres (D). Resident PC show thickening and abnormal arborisation (C, white arrowhead) of the dendrites. Concomitantly the EGL is cytodepleted and the granular layer becomes mildly disorganised. Calbindin-staining in the most affected hemispheres is restricted to scattered axons, loosely bundled in the foliary white matter (D, black arrowhead). PC perikarya in many folia are completely missing while the Purkinje cell layer features a moderate Bergmann´s gliosis. Scale bar: 0.7 cm for A; 130 μm for B,C,D.Genome-wide mRNA sequencing of a single canine cerebellar cortical degeneration case leads to the identification of a disease associated SPTBN2 mutation. Forman OP, De Risio L, Stewart J, Mellersh CS, Beltran E - BMC genetics (2012). Not Altered. CC.

Subacute cerebellar degeneration is characterized by the deterioration of the area of the brain concerned with muscle coordination and balance. 

There are two types of subacute cerebellar degeneration: 

  • Paraneoplastic cerebellar degeneration due to cancer
  • Thiamine deficiency associated cerebellar degeneration, caused by a lack of the vitamin B1 (thiamine) which may be related to alcoholism or a poor diet 

What is the Pathology of Subacute Cerebellar Degeneration?

Etiology: The cause of Subacute Cerebellar Degeneration is autoimmune. 

Pathogenesis: Autoimmune mediated  antibodies and T-cells attacking normal cells in the nervous systems.

How does Subacute Cerebellar Degeneration Present?

Patients with subacute cerebellar degeneration are typically over 50 years of age on average, and females are predominantly affected. The symptoms, features, and clinical findings associated with Subacute Cerebellar Degeneration include ataxia, dysarthria, dysphagia, diplopia, tremors, and vertigo. 

How is Subacute Cerebellar Degeneration Diagnosed?

The diagnostic criteria for paraneoplastic subacute cerebellar degeneration are:

  • Severe cerebellar symptoms less than 12 weeks, along with no abnormal sign of cerebellar size reduction
  • Rankin scale score of at least a 3, indicating that symptoms interfere with daily life significantly
  • Clinical evidence of cerebellar dysfunction, specifically within the trunk or hemispheres, and established diagnosis of cancer within 5 years from the onset of symptoms
  • Cerebellar symptoms and cancer-related antibodies

How is Subacute Cerebellar Degeneration Treated?

Subacute cerebellar degeneration is treated by prompt tumor removal, chemotherapy and radiation therapy. Adjuvant therapy with glucocorticoids such as methylprednisolone and immunotherapy with potent T cell inhibition, such as tacrolimus and rituximab may be helpful. Physical therapy and ocupational therapy should aso be considered. 

What is the Prognosis of Subacute Cerebellar Degeneration?

The prognosis of subacute cerebellar degeneration is poor. Most symptoms (such as ataxia and vision problems) can be reversed if detected and treated promptly. However, improvement in memory function and cognitive skills may be slow and, usually, incomplete. Without treatment, this condition can be disabling and life-threatening.

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Last updated on May 26, 2022