A Case of Linear Scleroderma Involving Cerebellum with Vertigo
A 17-year-old female patient visited our hospital for continuous spinning sense, which had been occurring for the past three days. The dizziness was continuous at rest, and aggravated with the moving head, accompanied by nausea and vomiting. The patient complained of left hearing loss that began approximately 1 year prior. There were no other otologicsymptoms, such as otalgia, ear fullness or otorrhea, etc. She had been taking Methotrexate (MTX) for linear scleroderma of the scalp since 2001, and had a history of undergoing resection and primary closure for progressive depression of the left forehead skin in 2010. Otoscopic examination of the ear showed that the external auditory canals and tympanic membranes in both ears were normal. Pure-tone audiometry showed sensorineural hearing loss of about 53 dB HL on the left side and normal hearing on the right side (). Tympanogram showed type A on both sides and stapedial reflex test was normal. In videonystagmography, spontaneous nystagmus toward the right side was observed (). The patient was admitted under suspicion for vestibular neuronitis and routine blood tests, electrocardiogram, and chest X-ray after admission were normal.\nIn positional nystagmus test, on the second hospital day, DCPN was observed. The nystagmus to the right side (25 deg/s) first appeared by rolling the head to the right in supine position, but when rolling the head to left, after about 58 seconds of right beating nystagmus (25 deg/s), the direction of nystagmuswas changed to the left side (20 deg/s), without changing the head position (). Saccade test, pursuit test and optokinetic test presented normal ranges, andbithermal caloric test showed the left canal paresis of 19% (). The neurologic and cerebral function exams were unremarkable. In cerebellar function tests, finger-to-nose test and heel-to-shin test were normal, but she showed instability in tandem gait test. Videonystagmography was performed again, and inhibition by visual fixation was not presented. With suspicion of central lesion, brain MRI and computed tomography were carried out. Multiple calcified lesions at the left cerebellum were observed. Compared with brain MRI in 2004, progressed lesions of the cerebellum were observed (). The cerebospinal fluid (CSF) study and eosinophil count study undergone for suspecting parasite infection were normal.\nThe patient was given a prescription for administration of antiemeticagent and antivertigo medication. Sixth day of admission, the patient was discharged because dizziness was improved. After two weeks, she still had the symptoms of dizziness, but it became mild and spontaneous nystagmus was not observed. At present, conservative treatment and observation is maintained.