Acute hemorrhagic leukoencephalitis (AHLE), is a rare inflammatory demyelinating disorder, variant of acute disseminated encephalomyelitis. The diagnosis of AHLE remains challenging due to the rarity of the disease and the lack of a reliable biomarker. We report here a case of a 73-year-old male patient with a progressive, low-grade febrile confusional syndrome 20 days after receiving the first dose of BNT162b2 vaccine against SARS-CoV-2.
View Article and Find Full Text PDFCerebrotendinous xanthomatosis (CTX) is a rare inherited disorder of the alternative pathway of bile acid biosynthesis, due to mutation(s) of the gene CYP27A1, leading to sterol 27-hydroxylase deficiency. The latter results in a systematic deposition of cholestanol and cholesterol to the central nervous system and tendons, premature cataract, as well as the manifestation of systematic symptoms, such as chronic diarrhea, osteoporosis, and premature atherosclerosis. Due to its marked clinical heterogeneity, prompt diagnosis of this disorder is challenging.
View Article and Find Full Text PDFA patient with a known history of hypothyroidism due to Hashimoto's thyroiditis presented with a subacute, progressive sensorimotor deficit that affected the upper limbs predominantly. The electrophysiological findings progressively evolved from multifocal motor conduction block to multifocal demyelinating sensory and motor nerve involvement with conduction block, and finally to findings fulfilling the diagnostic criteria of chronic inflammatory demyelinating polyneuropathy (CIDP). The patient did not respond adequately to intravenous immunoglobulin, whereas oral prednisone led to fast and complete recovery.
View Article and Find Full Text PDFEpidemiological data on the incidence of Bell's palsy (BP) are conflicting. The aim of this retrospective study was to evaluate the variation of cases of BP acutely presented in the emergency room of our department over a 15-year period (January 1990 to December 2004). We examined the monthly and seasonal distribution of BPs among 36,312 patients who came to our emergency services and compared it with the assumed equal distribution of cases over the year (null hypothesis) using chi(2) techniques.
View Article and Find Full Text PDFRickettsia conorii is endemic in the Mediterranean region. Infections are mostly benign and neurological involvement is unusual. We describe a case of a man who presented with acute facial nerve palsy followed by flaccid tetraparesis due to an electrophysiologically established polyneuritis with distal conduction failure.
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