Hemicerebral atrophy as an unusual central nervous system manifestation of Neurofibromatosis type 1: A case report

Neurofibromatosis type 1 is a widely known phakomatosis with highly variable manifestations. However, its presentation with hemicerebral atrophy resulting from an intracranial vasculopathy is uncommon, yet it poses a major health threat. We hereby report the case of a 6-year-old boy with the clinical features of Neurofibromatosis type 1 and imaging evidence of right internal carotid artery narrowing, with subsequent ipsilateral hemicerebral atrophy, after presenting with progressive left-sided body weakness and epilepsy. This case represents a rare manifestation within the vast imaging spectrum of Neurofibromatosis type 1, highlighting how unpredictable the condition can be.

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Publication Details

Journal
Radiology Case Reports
Published
2026-09-07
DOI
https://doi.org/10.1016/j.radcr.2026.08.017
Primary Topic
Neurofibromatosis and Schwannoma Cases
Type
article
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article

Hemicerebral atrophy as an unusual central nervous system manifestation of Neurofibromatosis type 1: A case report

Tinsae Zelalem Amare, Bethelhem Belachew, Hanan Tofik Ahmed, Yared Dejene Tefera et al.
Radiology Case Reports
Neurofibromatosis and Schwannoma Cases
article

Hemicerebral atrophy as an unusual central nervous system manifestation of Neurofibromatosis type 1: A case report

Tinsae Zelalem Amare, Bethelhem Belachew, Hanan Tofik Ahmed, Yared Dejene Tefera, Bethlehem Abera
article en

Abstract

Neurofibromatosis type 1 is a widely known phakomatosis with highly variable manifestations. However, its presentation with hemicerebral atrophy resulting from an intracranial vasculopathy is uncommon, yet it poses a major health threat. We hereby report the case of a 6-year-old boy with the clinical features of Neurofibromatosis type 1 and imaging evidence of right internal carotid artery narrowing, with subsequent ipsilateral hemicerebral atrophy, after presenting with progressive left-sided body weakness and epilepsy. This case represents a rare manifestation within the vast imaging spectrum of Neurofibromatosis type 1, highlighting how unpredictable the condition can be.

Radiology Case ReportsVol. 21(12)
St. Paul's Hospital Millennium Medical College (ET), Addis Ababa University (ET)
Good health and well-being
Openalex Percentile: Top 11%
Neurofibromatosis and Schwannoma Cases
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