Freiburg Neuropathology Case Conference

Excerpt

A 57-year-old patient had presented 5 years earlier with progressive bilateral hearing loss. At the time a magnetic resonance imaging (MRI) of the head had been performed, which excluded structural abnormalities of the inner ear as well as the presence of vestibular schwannoma. In retrospect, superficial siderosis of the brainstem and the cerebellum could be detected on the corresponding MRI (Fig. 1). Since the hearing loss progressed significantly the patient was treated with a left-sided cochlear implant (CI) 2 years later. Another 2 years later the patient was hospitalized in the department of neurology for vertigo, slight tremor as well as signs of ataxia, predominantly in the left leg. In addition, the patient complained of unexplained mood swings and progressive sleepiness. Among other clinical tests a lumbar puncture was performed which revealed the presence of subarachnoid blood. An MRI of the head had to be discontinued since the patient reported pain at the level of the CI when advanced into the gantry of the MR scanner. A computed tomography (CT) and CT angiography of the head were slightly distorted due to beam hardening artifacts related to the CI but revealed no subarachnoid blood or neurovascular pathologies (not shown). Whole body positron emission tomography with different radiotracers excluded signs of cerebellar multisystem atrophy, malignancies as well as a suspected cerebral amyloid angiopathy (not shown). Since additional MRI examinations of the spine could not be performed the patient underwent a Spinal CT after intrathecal administration of an ionic contrast agent which displayed dilated perimedullary veins (Fig. 2). A spinal catheter angiography revealed a hypertrophic radiculomedullary artery and a intraspinal tumor blush at the level L3 (Fig. 3). The patient underwent a spinal MR. This time the CI was firmly fixed with a bandage and the patient was positioned feet first into the gantry of a 1.5 T MR scanner. The MRI revealed a tumor at the level of L3 of about 1.5 cm in diameter. After interdisciplinary discussion, the indications for tumor resection were confirmed. Complete resection of the highly vascularized tumor was performed via a minimally invasive dorsal approach with extended right partial hemilaminectomy of L3. After initial postoperative delirium no new focal neurological deficits were observed and the postoperative course was uneventful. Cognition and gait disturbance improved significantly during rehabilitation.

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