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Washington University Experience | NEOPLASMS (GLIAL) | Diffuse midline glioma, H3 K27-altered | 5A0 Case 5 History
Case 5 History ---- The patient was a 4 year old black female, who was growing and developing normally until 15 months ago when she was observed to have a clumsy gait. Within two weeks this had progressed to include loss of appetite, reluctance to close her eyes, a dazed appearance with unclear speech and new onset enuresis. Family history was notable for a seizure disorder in a maternal aunt, hypertension, congenital heart disease and cancer. Bilateral abducens palsies were noted and opticokinetic nystagmus could not be elicited in either direction. The corneal reflexes were weak bilaterally with the left more than the right. Jaw strength was weak. Facial diplegia with left more than right was present. Hearing was intact to finger rub and whisper. Gag was intact. The tongue deviated to the left on protrusion. Sensory exam was intact with high level responses to medial extremity pinch. Motor exam revealed mildly to moderately increased tone in all four extremities. A CT scan with and without contrast showed a 2 cm circular mass in the junction of the left brachium pontis with the brainstern. It was contrast enhancing and surrounded by an area of hypodensity which involved virtually the entire brainstem and the lower reaches of the right cerebral peduncle. The fourth ventricle showed a slight decrease in size and the remaining ventricles were slightly increased. A repeat CT with high resolution of the posterior fossa with sagittal reconstruction was felt to be pathognomonic of a brainstem glioma. Therapy was begun administering 4800 rads to the thalamus, brainstem, cerebellum and upper cervical spine. Dexamethasone and prednisone were given to reduce edema and an improvement in facial strength and gait were noted. She was subsequently readmitted in June 1982 because of a deterioration related to steroid tapering. Her last admission was February 1983 because of increasing listlessness, headaches and decreased appetite. A repeat CT scan with and without contrast showed extension of the glioma and the patient was referred to Hematology/Oncology. Initial chemotherapy was begun with 2 courses of CCNU which was without result followed by VP-16. Neurologically she had progressed to quadriplegia. Her cranial nerve function had diminished notably in VI, VII, IX and X nerves. A repeat CT scan in mid-July 1983 showed an extensive lesion extending from just above the for amen magnum through the tentorial notch into the midbrain, c/w tumor recurrence. She was found dead at home at the end of July 1983
