Title : A Rare Case of Probable Neurosarcoidosis
Neurosarcoidosis is an uncommon manifestation and should be suspected in any patients with sarcoidosis with new neurological symptoms. We present a rare case of probable neurosarcoidosis, which was diagnosed by exclusion and elevated acetylcholinesterase (ACE) levels in the CSF.
A 62-year-old female with a history of HTN, DM, CHF, sarcoidosis, lung cancer (in remission), presented to the emergency department (ED) for seizure and encephalopathy. Vitals were unremarkable except for temperature of 100F and tachycardia 155 beats/min. Initial labs were unremarkable except for lactic acidosis 2.7 and Na 132. CT and MRI brain were unremarkable. Lumbar puncture was significant for WBC 1643 with 96% PMNs, glucose 51 and TP 153. We began treatment for bacterial meningitis with broad spectrum antibiotics, however all the subsequent testing for bacterial/viral/fungal suspects were negative. She also tested negative for RPR, HSV and HIV. The patient had to be upgraded to the ICU briefly as there were concerns that she was not able to protect her airway. It was discovered here that she was supposed to be on prednisone at home for sarcoidosis but non-compliance likely played a role. Her home dose of prednisone was resumed and after that, the patient began to improve drastically and she was discharged to a skilled nursing facility. The patient presented back to the ED three weeks later for encephalopathy and right sided hemiparesis. Initial labs and vitals on this presentation were unremarkable. MRI brain showed a subtle diffusion weighted signal and flair hyperintensity within the left parietal, left temporal and left occipital lobes. She underwent another lumbar puncture, which was remarkable for an elevated ACE level. Given the MRI and CSF findings, we made a diagnosis of probable neurosarcoidosis and initiated high dose steroids. She began to improve with the addition of steroids and was eventually discharged back to a skilled nursing facility with a steroid taper.
Neurosarcoidis is a rare form of sarcoidosis in which inflammation is deposited along any part of the CNS. Symptoms are dependent on the location of the inflammation. In our patient, these symptoms manifested as seizures, hemiparesis and encephalopathy. Biopsy is the gold standard of diagnosis, however obtaining one can be challenging. Therefore, many cases are diagnosed as probable neurosarcoidosis. To make this diagnosis, there needs to be high clinical suspicion, MRI/CSF findings suggestive of neurosarcoidosis and exclusion of all other pathologies. CSF ACE may be another tool to aid in the diagnosis of neurosarcoidosis. Although there is no consensus, there have been several studies done which indicate a strong correlation between elevated CSF ACE levels and neurosarcoidosis.
The gold standard for the diagnosis of neurosarcoidosis is a tissue biopsy which may be difficult to achieve. Therefore, neurosarcoidosis is a diagnosis of exclusion after all other pathologies have been ruled out.
What will audience learn from your presentation?
- This abstract highlights the importance of keeping neurosarcoidosis in the differential whenever a patient with known sarcoidosis presents with neurological symptoms.
- In the clinical setting, biopsy may be difficult to obtain to diagnose this condition; CSF ACE may be a helpful tool in such cases.
- This case also highlights the importance of compliance to steroids for those diagnosed with sarcoidosis