UNASSIGNED: We report a @entity1 of 68-year-old @entity1 who developed @entity1183 of feet in 2008. Ataxic gait disturbance, @entity2055 , @entity425 of lower limbs have gradually appeared and she couldn't walk without assistance in 2013. Her cognitive function declined subacutely in 2014. When she was admitted to our hospital, it was difficult to fully evaluate her @entity16 and cognitive function. The tendon reflex were absent and Babinski reflex showed positive in both sides of the lower limbs. Diffusion weighted image of MRI showed high intensity in cerebrocortical area, and variation @entity31967 @entity3669 gene mutation was detected. We diagnosed her with Gerstmann-Str  ussler-Scheinker (GSS) disease. Cerebellar symptom such as ataxic gait occurs as the initial manifestation in 90% of @entity1 with @entity24575 . Her initial symptom was @entity1183 of lower limbs and cerebellar symptom gradually appeared during the course of disease. In addition, her cognitive function declined six years after the onset. This case presented atypical clinical course as described above. Consequently, it led to diagnostic delay in @entity24575 .

A case of Gerstmann-Str  ussler-Scheinker disease presented with XXXX in the lower extremities.

@entity1 :: ('9606', 'Species') :: ['woman', 'patients', 'patient'] @entity1183 :: ('MESH:D006987', 'Disease') :: ['numbness'] @entity31967 :: ('p|SUB|P|102|L', 'ProteinMutation') :: ['P102L'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neurological symptoms'] @entity425 :: ('MESH:D018908', 'Disease') :: ['muscle weakness'] @entity3669 :: ('5621', 'Gene') :: ['prion protein'] @entity24575 :: ('MESH:D016098', 'Disease') :: ['GSS disease'] @entity2055 :: ('MESH:D001259', 'Disease') :: ['truncal ataxia']
@entity1183 :: (MESH:D006987,Disease) :: ['numbness']
