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Case 318: Adult Polyglucosan Body Disease
Eline Van den Borre1, Gert Cypers1, Piet Vanhoenacker1
1From the Department of Neurology, Onze-Lieve-Vrouwziekenhuis vzw, Moorselbaan 164, 9300 Aalst, Belgium (E.V.d.B., G.C.); Department of Radiology, Universitair Ziekenhuis Gent, Gent, Belgium (P.V.); and Department of Radiology, Universitair Ziekenhuis Antwerpen, Edegem, Belgium (S.D.).
History:
A 72-year-old man sought care for a cognitive deterioration over the past 5 years. There was a documented decline in his performance on the Mini-Mental State Examination (30 of 30 in 2016, 23 of 30 in 2021), with mainly episodic memory impairment. A more detailed history revealed a gait problem, paresthesia in both feet, and nocturnal urinary frequency. Clinical examination findings were suggestive of a length-dependent polyneuropathy. In addition, a right-sided Babinski sign was noted. Electromyography and a nerve conduction study corroborated a peripheral axonal sensorimotor neuropathy. MRI of the brain was performed.
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