Work

Teaching NeuroImage: Honeycomb Appearance of the Basal Ganglia Suggests Biallelic Nitrilase-1 Variants

Year: 2026

Type: article

Abstract: A previously healthy 37-year-old woman presented with daily headaches, episodic unsteadiness, sensation of swaying, and difficulty concentrating for 5 months.Within 5 years, she showed progressive cognitive decline (MoCA 19/30), paralleled by cerebellar ataxia and bradykinesia.Figure Brain MRI 5 Years Before, 1 Year, and 3 Years After Clinical Onset Brain MRI 5 years before (A and B), 1 year (C and D), and 3 years after clinical onset (E and F) demonstrate progressive symmetric periventricular leukoencephalopathy (outlined arrows) on FLAIR (A, C, E) and increasing honeycomb-like enlargement of perivascular spaces in the basal ganglia (filled arrows) on T2-weighted images (B, D, F).

Source: Neurology

DOI: 10.1212/wnl.0000000000214692

Authors: Tabea Zang, Tobias B. Haack, Michael Schlotterbek, Lena Zeltner, Ludger Schöls, Holger Hengel

Institutions: German Center for Neurodegenerative Diseases, Hertie Institute for Clinical Brain Research, University of Tübingen

Language: English

Topic: Neurological diseases and metabolism

Subfield: Neurology

Field: Neuroscience

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: bronze

OpenAlex ID: W7130395765

FWCI: 2.398

Cites: 2

Cited by: 1

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