BACKGROUND: Patients who develop progressive supranuclear palsy with predominant cerebellar ataxia (PSP-C) develop cerebellar ataxia as the initial and principal symptom, may be misdiagnosed as having multiple system atrophy with predominant cerebellar features (MSA-C). Therefore, we investigated the clinical signs and symptoms between PSP-C and MSA-C early in their disease course. METHODS: We reviewed the medical records of 15 consecutive patients with pathologically proven PSP-C (4) and MSA-C (11). We recorded the presence or absence of clinical features that developed within 2 years of disease onset. RESULTS: The age at onset of PSP-C patients was older than that of MSA-C patients (p = 0.009). The frequencies of falls were higher in PSP-C patients than in MSA-C patients (p = 0.026). Additionally, the development of supranuclear vertical gaze palsy was higher in PSP-C patients than in MSA-C patients (p = 0.011), whereas the frequency of dysautonomia was lower in PSP-C patients than in MSA-C patients (p = 0.035). CONCLUSIONS: Older onset, early falls, and supranuclear vertical gaze palsy without dysautonomia may predict the diagnosis of PSP-C in patients with late-onset sporadic cerebellar ataxia.
BACKGROUND:Patients who develop progressive supranuclear palsy with predominant cerebellar ataxia (PSP-C) develop cerebellar ataxia as the initial and principal symptom, may be misdiagnosed as having multiple system atrophy with predominant cerebellar features (MSA-C). Therefore, we investigated the clinical signs and symptoms between PSP-C and MSA-C early in their disease course. METHODS: We reviewed the medical records of 15 consecutive patients with pathologically proven PSP-C (4) and MSA-C (11). We recorded the presence or absence of clinical features that developed within 2 years of disease onset. RESULTS: The age at onset of PSP-Cpatients was older than that of MSA-C patients (p = 0.009). The frequencies of falls were higher in PSP-Cpatients than in MSA-C patients (p = 0.026). Additionally, the development of supranuclear vertical gaze palsy was higher in PSP-Cpatients than in MSA-C patients (p = 0.011), whereas the frequency of dysautonomia was lower in PSP-Cpatients than in MSA-C patients (p = 0.035). CONCLUSIONS: Older onset, early falls, and supranuclear vertical gaze palsy without dysautonomia may predict the diagnosis of PSP-C in patients with late-onset sporadic cerebellar ataxia.
Keywords:
Cerebellar ataxia; Falls; MSA-C; Multiple system atrophy; NINDS-SPSP; National Institute for Neurological Diseases and Stroke – the Society for Progressive Supranuclear Palsy; PSP; PSP-C; Progressive supranuclear palsy; Supranuclear vertical gaze palsy; multiple system atrophy with predominant cerebellar features; progressive supranuclear palsy; progressive supranuclear palsy with predominant cerebellar ataxia
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