Neurolist Case Study

Progressive Ataxia

14 y/o girl transferred from outside facility because of progressive ataxia. Patient states that symptoms started roughly 2-3 weeks ago after a basketball tournament where she was elbowed in the face that caused a mild lip abrasion. She states that since then, she has been experiencing constant, dull headache in the frontal area. She has also noticed dizziness accompanying these headahce and describes the sensation as if the room is spinning from left to right. She also described difficulty walking due to frequent falls and stated that the symptom had progressed to the point where she was unable to put on her own clothes without assistance and stayed mostly in her room. Other symptoms included mild photophobia, difficulty talking, slurred speech, but denied any difficulty with her language comprehension. Patient denied fever, chill, rhinorrhea, cough, nausea, vomiting, chest pain, or neck pain. Physical exam is significant for dysarthria and scanning speech, left deviation of uvula, bradykinesia of left upper and lower extremities but 5/5 strength in all extremities, normal DTRs, ataxia L > R, difficulty walking without assistance, wise based gait. No language comprehension difficulty, nystagmus, or neurocutaneous stigmata noted.  

A lumbar puncture was performed while patient was at the outside hospital with normal results. MRI without contrast at the outside hospital showed hyperintensity in the left cerebellar region. Given the history that the symptoms started after recent trauma to the face, arterial dissection remained a possibility and MRI with and without contrast was ordered for further evaluation. The repeat MRI showed increased signal intensity in the L cerebellum on DWI and was read by the radiologist as a stroke in the superior cerebellar artery distribution. MRA was ordered to rule out dissection and the result of the study was normal. Given the progressive worsening of her symptoms, the inital diagnosis of stroke was challenged and a MRI Spectroscopy was performed. The spectroscopy of the left cerebellar hemisphere showed choline elevation and N-Acetil aspartate depression, a pattern more consistent with tumor than stroke. Given this new information, the radiologist became concerned that the hyperintensity seen in the left cerebellar hemisphere is due to tumor rather than stroke. Patient was started on steroids and Neurosurgery was consulted for biopsy of the left cerebellar hemisphere for pathological study.

After the initiation of steroid, patient's symptom improved dramatically compared to that at the admission. Pathology study of the cerebellar biopsy demonstrated chronic meningoencephalitis of unknown etiology. Pathology stains are negative for CMV, herpes simplex, fungus, AFB, and bacteria. CSF PCR for mycoplasma, Epstein-Barr virus, HSV, and Enterovirus were negative. She was placed on seven day course of IV Doxycycline and her steroids were tapered off. 2 weeks after her admission to the hospital, patient was discharged home with imrpoving symptoms.