Gérald Glibert

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We describe an exceptional clinical picture, namely, cognitive impairment of the Alzheimer disease type in a man who later developed manifestations typical of amyotrophic lateral sclerosis and who was subsequently found to have adult polyglucosan body disease (APGBD) upon postmortem neuropathologic explorations. The combined occurrence of amyotrophic(More)
Hashimoto's encephalopathy (HE) is an infrequent disease with no well-known physiopathology. Status epilepticus is rarely reported in association with HE. We describe the 7-year evolution of a young woman who presented with recurrent status epilepticus as the main complication of HE. This evolution was especially marked by the occurrence of(More)
We describe a case of acquired hepatocerebral degeneration (AHD) presenting with confusion and worsening memory problems since her discharge from the gastroenterology units. Cases of AHD are rare and are frequently confused with hepatic encephalopathy and Wilson's disease. There are no proven pharmacological therapies for AHD. Information regarding the(More)
BACKGROUND Inclusion-body myopathy with Paget's disease of the bone and frontotemporal dementia (IBMPFD) is a rare, late-onset autosomal disorder arising from missense mutations in a gene coding for valosin-containing protein. CASE REPORT We report the case of a man carrying the previously described p.Arg159His mutation, who had an unusual axonal(More)
Clinical expression of POLG mutations is largely variable. We present a patient with a new mutation in spacer region of mitochondrial polymerase gamma protein (P765T). The clinical picture is characterized by the presence of sensory-ataxic neuropathy, ophthalmoplegia, dysarthria and gastroparesis, which had not been previously observed in ataxia neuropathy(More)
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