Frequent Falls -An Unusual Presentation of Sarcoidosis

Authors
Category Primary study
JournalAmerican Journal of Respiratory and Critical Care Medicine
Year 2022
Introduction -Sarcoidosis is an immune-mediated, granulomatous disorder that can affect any organ. Nervous system involvement occurs in approximately 5% of patients with sarcoidosis. We describe an unusual presentation of sarcoidosis with neurologic involvement causing panhypopituitarism without parenchymal lung disease. Case -A 65-year-old Caucasian man with a history of diabetes mellitus type II presented complaining of dizziness and falls. He described 3 weeks of lightheadedness upon standing, confusion, episodic falling, and a 20 pound unintentional weight loss. Vital signs demonstrated orthostatic hypotension, and his physical exam was remarkable for decreased sensation in his distal lower extremities. Initial evaluation included a computed tomography of the head, which demonstrated multiple lytic bone lesions, prompting further workup of potential metastatic disease. Cortisol testing revealed adrenal insufficiency that was treated with corticosteroids with improvement in his orthostatic hypotension. Subsequent endocrinologic testing demonstrated pan-hypopituitarism. To further evaluate for metastatic disease, a positron emission tomography-computed tomography was performed revealing bilateral, hilar lymph nodes with enhanced radiotracer uptake. Mediastinal lymph node biopsy performed via endobronchial ultrasound showed rare noncaseating granulomas. A lymph node biopsy was performed via mediastinoscopy, confirming the presence of noncaseating granulomas. Acid-fast bacillus and fungal cultures were unrevealing. A brain magnetic resonance image (MRI) displayed areas of enhancement involving the pituitary infundibulum, skull and dura, consistent with sarcoidosis. Lumbar puncture revealed oligoclonal bands, elevated protein, and lymphocytic pleocytosis. A multidisciplinary team concluded a probable diagnosis of neurosarcoidosis causing pan-hypopituitarism, and he was started on high-dose glucocorticoid therapy. The patient was later transitioned to infliximab infusions. Discussion -Common neurologic manifestations of neurosarcoidosis include cranial nerve palsies, peripheral neuropathy, myopathies, and headaches. In all patients with neurosarcoidosis, approximately half will present with neurologic symptoms as their initial complaint. Neurosarcoidosis with neuroendocrine involvement is the presenting symptom in only approximately 9% of patients with neurosarcoidosis. Neurologic evaluation in sarcoidosis is important as nervous system involvement is associated with significant morbidity. Definitive diagnosis is challenging, as it requires obtaining neural tissue for histologic analysis. Therefore, diagnostic criteria have been proposed for the probable diagnosis of neurosarcoidosis. These criteria suggest neurosarcoidosis as a diagnosis if the patient has histologic confirmation of systemic granulomatous disease along with MRI and cerebrospinal fluid analysis consistent with granulomatous inflammation of the nervous system. Typical treatment includes high-dose glucocorticoids. Infliximab therapy has been described in the literature as a possible treatment in those patients who fail to improve despite steroids.
Epistemonikos ID: b02e6a10b96b0a0600bc6489e64454463025f2dc
First added on: Feb 05, 2024