An English kindred with a novel recessive tauopathy and respiratory failure.

Nicholl, David J; Greenstone, Michael A; Clarke, Carl E; Rizzu, Patrizia; Crooks, Daniel; Crowe, Alex; Trojanowski, John Q; Lee, Virginia M-Y et al. · Ann Neurol · 2003

case_report · Level V

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Abstract

We present the clinicopathological features of two siblings from a consanguineous marriage who presented with respiratory hypoventilation and died 10 days and 4 years later, respectively. This disorder showed extensive tau neuropathology, and both had a novel homozygous S352L tau gene mutation. This is the first description of a pathologically proved young-onset tauopathy with apparent recessive inheritance.

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