Levodopa-responsive parkinsonism in hereditary spastic paraplegia with thin corpus callosum.

Abstract

Hereditary spastic paraplegia with thin corpus callosum is a rare degenerative disease, which is characterized by a progressive weakness of the lower limbs with a hypoplastic corpus callosum, and is often associated with other symptoms such as mental impairment, amyotrophy, sensory disturbances, dysuria, nystagmus and cataract. We describe two siblings (brother and sister) who showed a thin corpus callosum on MRI, one of whom showed the pure form of progressive spastic paraplegia, while the other showed predominant levodopa-responsive parkinsonism. The present cases are illustrative of a phenotypic heterogeneity in the same family of spastic paraplegia with a thin corpus callosum, despite the identical neuroimaging findings, and also presented another form of autosomal recessive juvenile levodopa-responsive parkinsonism.

• Authors: Suk Y Kang, Mee H Lee, Seung K Lee, Young H Sohn
• Journal: Parkinsonism & related disorders (Parkinsonism Relat Disord) Vol. 10 Issue 7 Pg. 425-7 (Oct 2004) ISSN: 1353-8020 [Print] England
• PMID: 15465400 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antiparkinson Agents
• Benztropine
• Selegiline
• Levodopa

Topics

• Adolescent
• Antiparkinson Agents (therapeutic use)
• Benztropine (therapeutic use)
• Cognition Disorders (etiology, physiopathology)
• Corpus Callosum (pathology)
• Electroencephalography
• Female
• Gait Disorders, Neurologic (etiology, physiopathology)
• Humans
• Levodopa (therapeutic use)
• Magnetic Resonance Imaging
• Male
• Muscle Weakness (etiology, physiopathology)
• Neuropsychological Tests
• Parkinson Disease (drug therapy, etiology)
• Selegiline (therapeutic use)
• Spastic Paraplegia, Hereditary (complications, pathology)
• Tremor (etiology, physiopathology)