Conclusion and Results (Outcome and follow-up)
Six weeks later, the patient presented to the hospital with the acute onset of left facial droop and worsening left-sided weakness. His vison by that time had progressed to almost complete visual loss. Neurological examination during this admission “hand motion” perception in both eyes, left facial droop, decreased left facial sensations, and spasticity in the left upper extremity (UE), with 0/5 power proximally and 1/5 distally, along with 3+ reflexes and a positive Hoffman sign. The right UE exhibited normal tone and 4/5 power. The left LE demonstrated increased tone compared to the right LE, and both LE weakness had progressed to grade 0 (no discernible contractions). Deep tendon reflexes were brisk bilaterally, with an absent ankle jerk and a negative Babinski sign. Sensations were absent in the bilateral LE and left UE. Code stroke was activated.
Non-contrast CT of the head and CT angiography of the head and neck showed no significant vessel occlusion, and the patient was not a candidate for IV thrombolysis due to being outside the treatment window. Stroke workup revealed an HbA1c of 5.1, and the lipid profile was normal. Cardiac telemetry showed no abnormal rhythm, and a transthoracic echocardiogram revealed normal left ventricular ejection fraction without evidence of a right-to-left shunt. MRI of the brain confirmed a new ischemic stroke with restricted diffusion in the right posterior limb of the internal capsule and adjacent thalamus. Additionally, new periventricular enhancement was observed in the right inferior lentiform nucleus, alongside unchanged corticomedullary junction T2 hyperintensities and nodular leptomeningeal enhancement at the brain base and brainstem, raising concerns for disease progression [Figure 5]. MRI of the cervical, thoracic, and lumbar spine remained stable with multiple areas of nodular leptomeningeal enhancement consistent with prior imaging.
As part of the workup for a potential diagnosis of neurosarcoidosis, a CT of the chest [Figure 6] revealed mediastinal lymphadenopathy and right hilar lymphadenopathy, raising the possibility of neoplastic disease or secondary sarcoidosis. Following a pulmonology consultation, bronchoscopy and endobronchial ultrasound-guided biopsy were performed, which showed reactive bronchial cells mixed with inflammatory cells, including histiocytes, but no definitive granulomas. Negative cytology did not entirely rule out malignancy or granulomatous disease. Additional testing, including an interferon-γ release assay for tuberculosis and an acid-fast bacilli (AFB) stain, was negative.
The patient was started on aspirin and a statin as a secondary prevention measure for this likely small vessel stroke. He was also started on high-dose methylprednisolone therapy (1 g/day), which led to improvements in vision, with return of color perceptions but still “hand motion” in acuity after two doses. A five-days course was completed. Upon discharge to an inpatient rehabilitation facility, the patient was prescribed prednisone 50 mg twice daily and mycophenolate mofetil 500 mg twice daily.
At the 3-month follow-up, there was subjectively increased leg strength, but no further improvement in vision. The patient continued to require a wheelchair for ambulation and experienced persistent loss of bowel and bladder function. MRI of the brain showed interval resolution of medullary involvement but revealed new leptomeningeal enhancement along the cavernous sinus. Consequently, a TNF-alpha inhibitor was initiated, with plans for follow-up in one month.