Teaching NeuroImages: MRI of brain findings of Wolfram (DIDMOAD) syndrome
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چکیده
منابع مشابه
Syndrome of the month Wolfram (DIDMOAD) syndrome
Wolfram syndrome (MIM 222300) is the association ofjuvenile onset diabetes mellitus and optic atrophy, also known as DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness). Patients present with diabetes mellitus followed by optic atrophy in the first decade, cranial diabetes insipidus and sensorineural deafness in the second decade, dilated renal outflow tracts early in t...
متن کاملThe Wolfram or DIDMOAD syndrome
Seven patients with a rare syndrome of diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy (OA), neurosensory deafness (D), atony of the urinary tract, and other abnormalities (Wolfram or DIDMOAD syndrome) are reported. Of the seven patients, three siblings were followed up for 10-17 years. All seven patients had diabetes mellitus and optic atrophy; six had diabetes insipidus; and in...
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A 29-week fetus was noted to have dilation of the entire ventricular system on prenatal ultrasound. Fetal MRI at 33 weeks revealed brain malformations suggestive of congenital muscular dystrophy (figure, A and B).1,2 Following normal delivery at 40 weeks, examination was remarkable for retinal detachment (figure, D) and optic nerve hypoplasia, without dysmorphic features. Sequence analysis show...
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A 74-year-old man, with well-controlled diabetes, presented with 3 months of progressive delirium. Examination revealed left homonymous upper quadrantanopsia. MRI demonstrated right temporal lesion and right lateral sinus thrombosis (figure). CSF examination revealed elevated protein level (1.31 g/L) without pleocytosis. Microbiology tests were negative, including HIV. Biopsy was performed and ...
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Gasperini syndrome is a rare crossed brainstem syndrome characterized by ipsilateral impairment of the VI, VII, and occasionally VIII cranial nerves and contralateral sensory loss. The syndrome, initially described by Ubaldo Gasperini in 1912, results from a lesion of the caudal pons tegmentum (figure e-1, links.lww.com/WNL/A47). The most frequent cause is the occlusion of the long circumferent...
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ژورنال
عنوان ژورنال: Neurology
سال: 2014
ISSN: 0028-3878,1526-632X
DOI: 10.1212/wnl.0000000000001082