Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, et al. A heterogeneous degeneration involving the brain stem basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. The hypothesized pathophysiology of cerebellar ataxia in PSP-C is also discussed.Ĭerebellar ataxia Multiple system atrophy Progressive supranuclear palsy Progressive supranuclear palsy atypical Spinocerebellar degeneration. In this review, we describe the clinical and neuropathological features of PSP-C.
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Furthermore, the clinical features of PSP-C have been identified, enabling it to be distinguished from other subtypes of PSP and MSA-C. Since the development of this classification, patients with PSP-C have been identified primarily in Asian countries, and it has been noted that this condition is very rare in Western communities. These patients can be classified as having PSP with predominant cerebellar ataxia (PSP-C), a new subtype of PSP.
However, more recently, it has been recognized that patients with PSP can present with truncal and limb ataxia as their initial symptom and/or main manifestation. As a result, pathologically proven PSP patients exhibiting cerebellar ataxia have often been misdiagnosed with spinocerebellar degeneration, specifically multiple system atrophy with predominant cerebellar ataxia (MSA-C). Cerebellar ataxia is one of the exclusion criteria given by the National Institute of Neurological Disorders and Stroke and the Society for Progressive Supranuclear Palsy. Progressive supranuclear palsy (PSP) is characterized by supranuclear gaze palsy, dystonic rigidity of the neck and upper trunk, frequent falls and mild cognitive impairment.
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