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.