Novel Presentation of Type 1 Wolfram Syndrome as Intracranial Hemorrhage and Longitudinally Extensive Transverse Myelitis: Neuroimaging and Angiographic Findings

Wolfram syndrome (WS), a rare genetic neurodegenerative condition, is primarily characterized by diabetes mellitus, optic atrophy, diabetes insipidus, and sensorineural hearing loss, along with diverse systemic manifestations. Late-stage cases exhibit widespread brain atrophy. While bleeding tendencies are not commonly observed in Type 2 WS (WS2), there is a distinctive bleeding tendency that is exclusive to WS2, with no documented occurrences in Type 1 WS (WS1). Extensive documentation exists regarding various neurologic manifestations of this syndrome; however, as of now, there is no reported mention of intracranial hemorrhage, a rarity within this condition.

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Publication Details

Journal
Annals of Indian Academy of Neurology
Published
2024-12-04
DOI
https://doi.org/10.4103/aian.aian_613_24
Primary Topic
Endoplasmic Reticulum Stress and Disease
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article
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article

Novel Presentation of Type 1 Wolfram Syndrome as Intracranial Hemorrhage and Longitudinally Extensive Transverse Myelitis: Neuroimaging and Angiographic Findings

Vikram Venkappayya Holla, H.R. Arvinda, S. K. Singh, SH Jayanth et al.
Annals of Indian Academy of Neurology
Endoplasmic Reticulum Stress and Disease
article

Novel Presentation of Type 1 Wolfram Syndrome as Intracranial Hemorrhage and Longitudinally Extensive Transverse Myelitis: Neuroimaging and Angiographic Findings

Vikram Venkappayya Holla, H.R. Arvinda, S. K. Singh, SH Jayanth, M Netravathi, KT Seetam
article en

Abstract

Wolfram syndrome (WS), a rare genetic neurodegenerative condition, is primarily characterized by diabetes mellitus, optic atrophy, diabetes insipidus, and sensorineural hearing loss, along with diverse systemic manifestations. Late-stage cases exhibit widespread brain atrophy. While bleeding tendencies are not commonly observed in Type 2 WS (WS2), there is a distinctive bleeding tendency that is exclusive to WS2, with no documented occurrences in Type 1 WS (WS1). Extensive documentation exists regarding various neurologic manifestations of this syndrome; however, as of now, there is no reported mention of intracranial hemorrhage, a rarity within this condition.

Annals of Indian Academy of NeurologyVol. 28(2)
National Institute of Mental Health and Neurosciences (IN)
Good health and well-being
Openalex Percentile: Top 77%
Endoplasmic Reticulum Stress and Disease
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Novel Presentation of Type 1 Wolfram Syndrome as Intracranial Hemorrhage and Longitudinally Extensive Transverse Myelitis: Neuroimaging and Angiographic Findings — Vikram Venkappayya Holla, H.R. Arvinda, et al. · Annals of Indian Academy of Neurology (2024) | TGRS Research Map | TGRS