Teaching Video NeuroImage: Perivascular Tumefactive Neurosarcoidosis of the Basal Ganglia

Case Report

A 65-year-old man presented with dysarthria evolving to lingual dyskinesia and chorea (Video 1). MRI brain demonstrated bilateral caudate nuclei and putamina perivascular enhancement with overlying T2/fluid-attenuated inversion recovery hyperintensity and ventricular compression (Figure). Spinal cord MRI was unremarkable. Whole-body PET/CT was negative for cancer and systemic inflammation. CSF revealed elevated proteins (74 mg/dL), 3/μL nucleated cells, and no oligoclonal bands. CSF infectious testing, cytology, flow cytometry, and comprehensive serum/CSF neural antibody testing were negative. Serum soluble interleukin-2 receptor was 1,122 pg/mL (≤858.2 pg/mL). Caudate head biopsy revealed nonnecrotizing granulomas with CD68⁺ multinucleated giant cells and infiltrating perivascular CD3+/CD20+ lymphocytes, consistent with neurosarcoidosis. Tissue-based fungal and acid-fast bacilli testing was negative. High-dose corticosteroids resulted in clinicoradiologic improvement, with reduced dysarthria and chorea. Infliximab was used as a steroid-sparing strategy. Intraparenchymal neurosarcoidosis is often perivascular and may have a tumefactive appearance.¹ Isolated extrapyramidal neurosarcoidosis is rare and can mimic lymphoma, vasculitis, CLIPPERS, or autoimmune GFAP astrocytopathy.²

Figure. MRI Showing Tumefactive Extrapyramidal Neurosarcoidosis.

T1 gadolinium-enhanced MRI sequences (A.b, A.d, A.f, A.h) demonstrated symmetric perivascular speckled enhancement. T2/FLAIR MRI sequences (A.a, A.c, A.e, A.g) revealed overlying hyperintensity of the bilateral caudate head and putamina with ventricular narrowing. Posttreatment T1 gadolinium-enhanced (B.b, B.d, B.f, B.h) and T2/FLAIR (B.a, B.c, B.e, B.g) sequences showed substantial improvement following 12 weeks of IV solumedrol. FLAIR = fluid-attenuated inversion recovery.

Video 1

Choreiform movements on examination. Clinical examination revealed chorea of the head, face, torso, and upper extremities with extensor posturing of the right fifth digit. *Note: The patient is not actively speaking during this video; his facial movements are unintentional.Download Supplementary Video 1 ^{(5.3MB, mp4)} via http://dx.doi.org/10.1212/209899_Video_1

Author Contributions

A. Aboseif: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data. N.P. Young: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data. A. McKeon: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data.

Study Funding

No targeted funding reported.

Disclosure

A. Aboseif and N.P. Young report no relevant disclosures. A. McKeon reports grants from the NIH (grants RO1NS126227 and U01NS120901) during the conduct of the study, consulting fees from Janssen and Roche (all paid to Mayo Clinic) outside the submitted work, and had a patent for MAP1B antibody issued, a patent for Septins 5, 7, GFAP, PDE10A, KLHL11, CAMKV antibodies pending, and a patent for Septin antibodies and MAP1B antibodies with royalties paid. Go to Neurology.org/N for full disclosures.