Teaching NeuroImages Neurology Resident and Fellow Section

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Teaching NeuroImages Neurology Resident and Fellow Section A 31-year-old patient with type-1 diabetes mellitus, hypothyroidism, visual and hearing loss Teaching NeuroImages Neurology Resident and Fellow Section © 2013 American Academy of Neurology

Vignette 31-year-old female, previously diagnosed with Wolfram syndrome At the age of 5, she was diagnosed with type 1 diabetes mellitus At the age of 16, she was diagnosed with hypothyroidism She has suffered from progressive visual loss and optic atrophy for the past 12 years She developed progressive hearing loss 3 years ago Abdominal sonography revealed findings consistent with neurogenic bladder and bilateral hydronephrosis Gocmen R, et al. © 2013 American Academy of Neurology

Imaging A. Axial T2W MRI image shows atrophy of the brainstem and middle cerebellar peduncles. B. Sagittal T1W image demostrates absence of the neurohypophyseal 'bright signal' (arrow) and atrophy of the brainstem and cerebellum. C. Axial T2W image shows hyperintense signal alterations at ventral part of pons (arrow). Gocmen R, et al. © 2013 American Academy of Neurology

Imaging A. Axial FLAIR image demonstrates increased signal intensity in bilateral peritrigonal areas/optic tracts (arrows). B. Coronal T2W image shows thinning/atrophy of bilateral optic nerves (arrows). Gocmen R, et al. © 2013 American Academy of Neurology

MRI of Brain Findings of Wolfram Syndrome Wolfram syndrome, which was first described in 1938, is a rare autosomal recessive disorder (1). It features diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy (OA), and deafness (D) (DIDMOAD Syndrome). It is caused by a mutation in the WFS1 gene that encodes wolframin, a transmembrane protein of pancreatic β cells. The life expectancy of patients diagnosed with this syndrome is about 30 years. Neuroimaging findings: Absence of the neurohypophyseal 'bright signal' on T1W images, atrophy of optic nerves and chiasm and tracts, atrophy of hypothalamus, brainstem, cerebrum, and cerebellum (2). Teaching point: The diagnosis of Wolfram syndrome is primarily based on clinical findings. However, interpretation of imaging findings is essential. If you see above imaging findings with the clinically relevant findings, think of DIDMOAD syndrome. REFERENCES Scolding NJ, Kellar-Wood HF, Shaw C, Shneerson JM, Antoun N. Wolfram syndrome: hereditary diabetes mellitus with brainstem and optic atrophy. Ann Neurol 1996;39:352–360. 2. Ito S, Sakakibara R, Hattori T. Wolfram syndrome presenting marked brain MR imaging abnormalities with few neurologic abnormalities. AJNR Am J Neuroradiol 2007;28:305–306. Gocmen R, et al. © 2013 American Academy of Neurology