New insights into the pathoanatomy of spinocerebellar ataxia type 3 (Machado-Joseph disease)
- Curr Opin Neurol. 2008 Apr;21(2):111-6. doi: 10.1097/WCO.0b013e3282f7673d.
- 1. Institute for Clinical Neuroanatomy, J. W. Goethe-University, D-60590 Frankfurt/Main, Germany. [email protected]
Purpose of review: This review summarizes recent neuropathological findings in spinocerebellar ataxia type 3 and discusses their relevance for clinical neurology.
Recent findings: The extent of the spinocerebellar ataxia type 3 related central nervous neurodegenerative changes has been recently systematically investigated in a series of pathoanatomical studies. These studies showed that the extent of the central nervous degenerative changes of spinocerebellar ataxia type 3 has been underestimated so far. The newly described pattern of central nervous neurodegeneration includes the visual, auditory, vestibular, somatosensory, ingestion-related, dopaminergic and cholinergic systems. These pathological findings were correlated with clinical findings and explain a variety of the spinocerebellar ataxia type 3 symptoms observed in clinical practice.
Summary: Systematic pathoanatomical analysis of spinocerebellar ataxia type 3 Brains helps to understand the structural basis of this Neurodegenerative Disease and offers explanations for a variety of disease symptoms. This better understanding of the neuropathology of the condition has implications for the treatment of spinocerebellar ataxia type 3 patients and represents a basis for further biochemical and molecular biological studies aimed at deciphering the pathomechanisms of this progressive ataxic disorder.