Importance Primary neurolymphomatosis is a rare presentation of non-Hodgkin lymphoma characterised by initial peripheral nervous system involvement without systemic disease, frequently resulting in delayed diagnosis. Observations A 56-year-old man presented with a 5-year history of asymmetric progressive radiculoplexoneuropathy, initially treated with immunotherapy for presumed vasculitic neuropathy, with apparent disease stability for 4.5 years before rapid deterioration over the ensuing 6 months. Examination revealed asymmetric limb weakness of lower and then upper limbs, areflexia, distal sensory loss and cranial nerve palsies. Magnetic resonance imaging demonstrated diffuse thickening and enhancement of the brachial and lumbosacral plexuses and cauda equina. Fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET-CT) showed hypermetabolic lesions involving the left sciatic nerve and cauda equina without systemic disease. Cerebrospinal fluid cytology and flow cytometry confirmed high-grade B-cell lymphoma. High-dose methotrexate–based combination chemotherapy achieved clinical remission at 14-month follow-up. Conclusions and Relevance: Chronic atypical radiculoplexoneuropathy with accelerated progression should prompt consideration of neurolymphomatosis to facilitate early diagnosis and improved outcomes.
Wernicke encephalopathy is classically associated with signal changes involving the mamillary bodies, medial thalami, and periaqueductal gray matter on MRI. We present a case of a 57-year-old woman with progressive Wernicke encephalopathy with imaging abnormalities in the cerebellar vermis and bilateral cerebellar hemispheres. This case highlights atypical imaging features that can be seen in Wernicke encephalopathy and emphasizes the importance of early clinical recognition to prevent progression.
A 62-year-old woman presented with left-sided facial and intraoral sensory disturbance due to a relapse of aquaporin-4 immunoglobulin G–positive neuromyelitis optica spectrum disorders (NMOSD). Brain MRI demonstrated an isolated lesion in the left extramedullary trigeminal nerve root. Although various diseases should be considered in the differential diagnosis of isolated trigeminal neuropathy, the trigeminal nerve root may represent a target during NMOSD relapses. This case highlights an unusual extramedullary trigeminal nerve root lesion as a potential manifestation of NMOSD.
A 48 year-old female with a history of metastatic lung adenocarcinoma to the brain presented with acute onset dizziness for 12 hours. The neurological exam was notable for subtle sustained nystagmus on right and upward gaze. A code stroke was called and CT brain revealed no acute intracranial abnormality. MRI brain demonstrated new subtle restriction diffusion and FLAIR hyperintensity along the anterior surface of the brainstem and the middle cerebellar peduncles without evidence of enhancement, concerning for leptomeningeal disease. The remainder of the supratentorial metastases were stable. Lumbar puncture was performed and CSF analysis confirmed metastatic adenocarcinoma.
We report an infant with HSV-1 encephalitis presenting with multifocal extra-temporal hemorrhagic lesions and complete temporal lobe sparing on neuroimaging. This case highlights the importance of considering herpes simplex encephalitis and initiating empirical acyclovir therapy even in the absence of the characteristic temporal pattern.
