Labrune’s Syndrome Presenting With Stereotypy-Like Movements and Psychosis: A Case Report and Review

Labrune’s syndrome, or leukoencephalopathy with brain calcifications and cysts (LCC), is a rare genetic syndrome with variable neurological presentations. Psychiatric manifestations and involuntary movements are uncommonly reported. We report the case of a 19-year-old female, initially diagnosed with Fahr’s syndrome, who presented to us with acute psychosis, abnormal behavior and involuntary movements. Her brain computed tomography showed extensive bilateral intracranial calcifications without cysts. Genetic testing detected two compound heterozygous variants, NR_033294.1 n.*9C>T and n.24C>T, in the SNORD118 gene, confirming the diagnosis of LCC. We discuss the expanding phenotypic spectrum of LCC and provide a literature review on the current diagnosis and management of this rare syndrome.

Central nervous system intravascular lymphoma in a Malaysia

Intravascular lymphoma (IVL) is a rare subtype of extranodal diffuse large cell lymphoma, characterized by intravascular proliferation of B or T lymphocytes within small blood vessels; which may lead to occlusive symptoms, its neurological involvement has been said to be uncommon among Asians.1 We describe a Malaysian with central nervous system IVL, to demonstrate that IVL is an important differential diagnosis in diffuse brain pathology also among Asians.