A 73-year-old wheelchair-bound male, with a history of chronic right frontal lobe infarct with residual left-sided weakness, atypical parkinsonian syndrome, three-vessel coronary artery disease, non-ST segment elevation myocardial infarction, hypertension, hyperlipidemia, and neurogenic bladder, presented with weakness, imbalance, and gait disequilibrium beginning in 2008. Brain magnetic resonance imaging in January 2008 was remarkable for leptomeningeal and nodular enhancement in both cerebral hemispheres involving the frontal and parietal lobes, suggestive of frontoparietal pachymeningitis. He was also found to have midcervical myelopathy and underwent cervical spine laminectomy in 2009. There was some question of a dural fistula of the superior sagittal sinus, but a cerebral angiogram was negative for vascular malformation. An outside lumbar puncture in 2008 was reportedly normal. In 2010, the patient had an extensive workup including a lumbar puncture and serology, which was negative. Cerebrospinal fluid studies included Gram stain, fungal culture, acid-fast stain, tuberculosis culture, malignant cell cytology, serum protein electrophoresis, immunoglobulin G index, oligoclonal bands, myelin basic protein, cryptococcal antigen, and viral panel (herpes simplex virus, arbovirus, California encephalitis virus, Saint Louis encephalitis virus, western equine encephalitis virus, eastern equine encephalitis virus, and West Nile virus). Serology studies included the complete blood count with differential, angiotensin-converting enzymes, immunoglobulin G typing, erythrocyte sedimentation rates, C-reactive protein, antinuclear antibodies, cytoplasmic antineutrophil cytoplasmic antibodies, perinuclear antineutrophil cytoplasmic antibodies, atypical anti-neutrophil cytoplasmic antibodies, Lyme titers, rheumatoid factor, rapid plasma reagin, venereal disease research laboratory tests, viral cultures, and Sjogren's syndrome antibody testing. 24-hour urinary copper was also normal. Electromyography showed the right carpal tunnel and fifth lumbar radiculopathy. The patient declined a meningeal biopsy at that time and was given the presumptive diagnosis of idiopathic pachymeningitis.
Following this workup in 2010, the patient had interval brain magnetic resonance imaging which was relatively stable but with some fluctuations in the leptomeningeal enhancements which, according to his daughter, appeared to correlate with clinical fluctuations in weakness and mobility. The patient's functional status gradually worsened from 2010 to 2012, as he was no longer able to drive or work in 2010. In 2011, he began having difficulty walking without a handrail and had intermittent falls once per year. He was treated at an outside hospital for a right frontal lobe stroke with residual left-sided weakness in 2012. Magnetic resonance imaging on presentation to our hospital in 2020 showed chronic deep white matter infarct in the right frontal lobe. The patient was also diagnosed with an atypical parkinsonian syndrome in 2013 and had follow-up at our Parkinson's and Movement Disorders Center through 2020, due to his neurologic examination showing flat affect, fluent but dysarthric speech, ideomotor apraxia, moderate hypomimia, mildly diminished downward ocular saccades, neck rigidity, asymmetric left-predominant bradykinesia, and postural instability. The patient was trialed on multiple treatments, including pramipexole, carbidopa-levodopa, and ropinirole without much improvement in his parkinsonian symptoms. He ultimately became wheelchair-bound in 2019.
During hospitalization for sepsis and cardiogenic shock in January 2020, repeat brain magnetic resonance imaging showed further interval increase in the size and number of multiple heterogeneously enhancing dural base and leptomeningeal lesions compared to brain magnetic resonance imaging performed in October 2019 at an outside hospital (Figure 1). Of note, outside October 2019 imaging also showed an interval increase in leptomeningeal nodular enhancement compared to prior imaging in 2017. The lumbar puncture was unremarkable. Magnetic resonance imaging of the cervical and thoracic spine showed only some age-related changes. Chest computerized tomography did not show any abnormalities related to possible sarcoidosis. The patient subsequently underwent a brain biopsy in September 2020, which showed granulomatous pachymeningitis without necrosis, suggestive of neurosarcoidosis. He then received treatment with 3 months of prednisone 60 mg daily from October 2020 to January 2021, discontinued after lack of symptomatic improvement in his weakness and mobility in addition to development of leg swelling. Further treatment with dexamethasone and disease-modifying therapy was offered. However, after discussion, the patient, his family, and the care team ultimately agreed not to proceed given his comorbidities and age.