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Dimitri Renard, MD; Laurent Collombier, MD; Giovanni Castelnovo, MD; Anouck Remy, MD; Jean-Louis Laplanche, PhD; Pierre Labauge, MD, PhD

Arch Neurol. 2008;65(10):1390-1391.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

A 52-year-old right-handed woman presented with progressive memory difficulties and fatigue for 6 weeks. Her mother had died at the age of 46 years, after 6 months of progressive cognitive impairment. Clinical examination showed an apathic, depressed patient with cerebellar ataxia, supranuclear upgaze palsy, episodic memory impairment, and semantic paraphasias. Mini Mental State score was 19 of 30. Neuropsychological testing showed loss of spontaneity, poor verbal fluency tests, impaired forward and backward digit span, and anterograde amnesia with both recall and recognition difficulties, together with moderate agraphia and ideomotor apraxia. There were no hallucinations. Brain magnetic resonance imaging showed basal ganglia and cortical hyperintensities on fluid-attenuated inversion recovery and diffusion-weighted imaging (Figure 1A-D). Electroencephalography revealed generalized periodic sharp wave complexes with left predominance (Figure 1E). Cerebrospinal fluid . . . [Full Text of this Article]

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