Behçet’s Disease
Patient Presentation
Patient C.O., a 27 year old male presented to an outside hospital with increased lethargy per family for the past month, right sided weakness that has progressed to the point of difficulty ambulating, and diplopia. The patient has a history of Behçet's disease that was diagnosed 3 months prior while the patient was incarcerated, as well as a seizure disorder. The patient admits to being non-compliant with his home medications, prednisone and Keppra®. At the outside hospital a CT scan was done, where they found a left thalamic and midbrain hypo-density. He was transferred to a higher level of care facility for a neurosurgery consultation. A full set of labs were sent including a CMP and blood cultures x2. 12-lead EKG and chest x-ray were obtained at the bedside. The patient is lethargic, but is alert and oriented and follows commands on all extremities. He is weak, and is urinating on himself. He is complaining of double vision and neck/headaches. He has painful open wounds in his mouth and genitalia. An MRI is ordered to further evaluate the hypo-density.
Differential List
It is possible that the hypo-density is a basal ganglia mass or tumor. The MRI will be able to differentiate between mass and neuro-Behçet's. Due to the stiffness and neck pain we also must rule out meningitis. Other possibilities are neuro lymphoma or a demyelinating lesion.
Diagnosis
Once the patient was admitted, C.O. received an MRI. The radiologist reported a left thalmus and midbrain enhancement on the T2 axial flair study, with mild mass effect, mild hemorrhage and midline shift. The enhancement was consistent with neuro-Behçet's. A lumbar puncture was done at the bedside, which resulted in a cloudy CSF with WBC of 249 (elevated), also consistent with neuro-Behçet's. Rheumatology was consulted to manage Behçet's. Two sets of blood cultures were negative.
Treatment
Over a week course the patient was placed on intravenous Solu-Medrol®, then to PO prednisone. We increased his home dose of 10mg of prednisone once a day, to 60mg prednisone once a day for the neuro-Behçet's advancement. With the evidence of a neuro lesion and positive LP that showed protein and WBCs in the CSF, the prognosis of 3 year survival is at 10%. The patient had a Hemoglobin A1C level of 7.7 on admission, so along with steroid treatment we started him on a sliding scale of insulin. Continue his at home seizure prophylaxis of 500mg of Keppra®, after EEG results showed that the patient was not having any active seizures. Consult Physical and Occupational therapy for a gait evaluation and recommendations.
Outcome
Patient continued Physical Therapy at an acute rehabilitation facility for gait training as well as continued medications. The double vision was likely caused by Behçet's ocular disease which affects 25-75% of patients. The patient should follow up with ophthalmology as this will result in vision loss if not tended to. The damage is not reversible; however the progression can be slowed with aggressive therapy.
Case created by Courtney Oliver, 2012.