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Background and objective: Spinocerebellar ataxia 7 (SCA7) is characte rised by cerebellar ataxia and visual loss. The aim of the present study was to elucidate the magnetic resonance ima ging (MRI) findings characteristic of patients with SCA7. Methods: Twenty patien ts with SCA (eight SCA3, three SCA6, and nine SCA7) and 20 control subjects unde rwent an MRIbased volumetric analysis. Results: The pontine volume in patients w ith SCA7 was decreased by a greater amount than in patients with other types of SCA (p < 0.01), whereas the cerebellar volume was not different from that in oth er types of SCA (p > 0.05). Pontine atrophy was a consistent finding in all pati ents with SCA7 regardless of the degree of cerebellar atrophy or the severity or duration of illness. In contrast, cerebellar atrophy was not found in those wit h a short duration of illness or mild ataxia, but became prominent as the severi ty and duration of illness progressed. Conclusions: Our study suggests that neur odegeneration is ongoing during the life of individuals with SCA7, and that the primary pathology in these individuals involves the brainstem rather than the ce rebellum. In addition, pontine atrophy is a prominent, consistent finding in SCA 7, and may help in establishing the clinical diagnosis of SCA7.
Background and objective: Spinocerebellar ataxia 7 (SCA7) is characte rised by cerebellar ataxia and visual loss. The aim of the present study was to elucidate the magnetic resonance ima ging (MRI) findings characteristic of patients with SCA7. Methods: Twenty patien ts with Results: The pontine volume in patients w ith SCA7 was decreased by a greater amount than in patients with other types of SCA (p & lt; 0.01), while the cerebellar volume was not different from that in oth er types of SCA (p> 0.05). Pontine atrophy was a consistent finding in all pati ents with SCA7 regardless of the degree of cerebellar atrophy or the severity or duration of illness of In contrast, cerebellar atrophy was not found in those wit ha short duration of illness or mild ataxia, but became prominent as the severi ty and duration of illness progressed. Conclusions: Our study suggests that neur odege neration is ongoing during the life of individuals with SCA7, and that the primary pathology in these individuals involves the brainstem rather than the ce rebellum. In addition, pontine atrophy is a prominent, consistent finding in SCA 7, and may help in establishing the clinical diagnosis of SCA7.