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Washington University Experience | MISCELLANEOUS | Neurosarcoid - no PNS | 7A0 Case 7 History
Case 7 History ---- This 50 -year-old woman had a past medical history of hypertension and Type II diabetes mellitus with retinopathy, neuropathy and a neurogenic bladder. She described tremor, occasional falls, intermittent slurred speech and periodic posterior neck pain with radiation to the temporal regions. Imaging studies at that time revealed diffuse leptomeningeal enhancement of the central nervous system, including the spinal cord. Lumbar puncture did not reveal an infectious etiology for this enhancement.
She was treated with a course of steroids, and a follow-up MRI showed some improvement in but not normalization of the enhancement. Abdominal and chest CT scans were negative and ACE (serum and CSF) levels were normal. Electromyography showed bilateral lumbosacral polyradiculopathy as well as sensory motor neuropathy. The patient’s final admission was due to a two day history of lethargy, speech difficulty and confusion. She also complained of headache and dysphagia. MRI scans revealed new lacunar infarctions in the subcortical white matter but was otherwise unchanged. She had a slowly declining neurological course with progressive lethargy and obtundation. General autopsy revealed the immediate cause of death to be bilateral acute pulmonary thromboembolism.
The fixed brain weighed 1410 grams and the leptomeninges were thickened and cloudy. The lacunar infarcts were confirmed, in the left globus pallidus, the left putamen and the left corona radiata. Microscopic examination revealed extensive chronic granulomatous inflammation of the leptomeninges with dissection into the Virchow - Robin spaces. Similar granulomatous inflammation was identified in neocortex, brainstem, cerebellum, spinal cord, spinal nerve roots, cranial nerves, and pituitary gland. Beside the CNS, granulomatous inflammation was also identified within all lymph nodes sampled (mediastinal and para-aortic), as well as in the spleen. Special stains for fungi (GMS) and mycobacteria (AFB) were negative (as was PCR for Mycobacterium tuberculosis).
