A 40 year old woman was presented to Shariati hospital (TUMS), with headache, nausea, and episodic amnesia. The episodes lasted less than five minutes, and resolved after vomiting.
The patient complained of no fever, focal weakness, or sensory symptoms; but she mentioned episodic dyspnea for more than 5 years, leading to cardiac and respiratory evaluations, which failed to show any specific etiology.
She had a history of admission to other hospital, about 9 months earlier, due to headache, blurring of vision and diplopia. At that time she was diagnosed as cerebral venous sinus thrombosis and had been treated with anticoagulants.
Examination disclosed blurred optic disc margins and slight neck rigidity. Brain MRI showed diffuse leptomeningeal enhancement, with scattered white matter changes (Figure 1).
Cerebrospinal Fluid (CSF) examination showed lymphocytosis (WBC:37,70% lymphocyte), elevated protein (277mg/dl) and low glucose (13mg/dl); but cytological examination, stains for bacteria, mycobacteria, and fungi, VDRL, Wright, and PCR for TB, all were negative in the CSF.
CSF exam was repeated three more times, each time with complete laboratory evaluation (including cytological examination), with similar results.
Routine hematology and biochemistry tests, liver function tests, thyroid function tests, PPD (purified protein derivative), HIV antibody, C-ANCA, P-ANCA, C3, C4, CH50, anticardiolipin and antiphospholipid antibodies, were normal/negative, as well as tumor markers in search of malignancy. Chest roentgenography, HRCT of thorax, and the result of bronchoscopy with BAL (BronchoAlveolar Lavage) were unrevealing.Serum ACE level was normal, but CSF ACE was raised (24; NL < 5).
The patient was treated with IV methyl prednisolone and continued on prednisolone with the impression of probable neurosarcoidosis.
Two weeks later, she developed another episode of dyspnea. She was re-evaluated by pulmonary specialist, and because of an abnormality in the base of her lung, an open biopsy was performed. The pathology report was consistent with Sarcoidosis. Dyspnea was ascribed to pulmonary hypertension.
Patient's neurological symptoms and signs improved after 2 months and after 7 months follow up, she was still free of symptoms.