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Washington University Experience | NEOPLASMS (HEMATOLYMPHOID) | Lymphoma, primary | 6A0 Case 6 History
Case 6 History ---- This 16 year old patient was in his usual state of good health until 3/19 when he developed URI symptoms which lasted about a week. One week later, he developed pounding intermittent frontal headaches that occurred several times a day. By 3/15, he was having frequent episodes of emesis and arm and leg weakness. MRI on 3/31 demonstrated white matter changes in the deep nuclei. LP showed an opening pressure greater than 36 cm of water, protein 41, glucose 61, RBC 43, WBC 44 and 95% lymphs. Oligoclonal bands were negative. CSF cultures were negative. After 1 day of Solumedrol he showed substantial improvement. He was felt to have a central demyelinating process which was steroid responsive. He was discharged home on 4/21 on a steroid taper. Several weeks later he noticed left-sided numbness and severe headache. On route in the ambulance he arrived unresponsive with a dilated right pupil and flexor posturing of his right side. He also had Cheyne-Stokes respirations. A head CT reportedly revealed cerebral edema and thus he was felt to be herniating, was intubated, hyperventilated and given Lasix, Mannitol and Decadron. During Decadron tapering he worsened and he was admitted to SLCH on 5/12. Neuro exam on admission to SLCH was remarkable for bilateral papilledema and left retinal hemorrhage. Included in the differential were: ADEM, MS, Schilder’s disease, adrenal leukodystrophy, and lymphoma. A head CT on 5/15 revealed extensive white matter lucencies. There was mass effect with midline shift to the left. The patient continued to require Decadron. A neurosurgical consult was obtained for a brain biopsy. Angiography prior to biopsy showed normal vasculature. Biopsy was planned for 5/17. However on 5/16 the patient had a decrease in responsiveness. The patient was given Lasix and Mannitol and he became more responsive with this intervention and a repeat CT was done. At the CT scanner, the patient again became unresponsive and developed unequal pupils. CT revealed no change from previous study. By 5/17 the patient had improved such that he was arousable and following commands. A biopsy of the right hemisphere white matter lesion was done showing lymphoma. He required aggressive treatment with Lasix, Mannitol, Decadron and frequent hyperventilation. He continued to have fluctuations in his neuro exam including a dilated left pupil on and off as well as a mild left hemiparesis on and off. It was felt that the patient could not tolerate chemotherapy or XRT because of his frequent episodes of increased intracranial pressure. For that reason, both of these therapeutic regimens were not initiated immediately, but XRT was eventually initiated. He tolerated the first few doses of XRT but by the third he was lethargic and groaned when stimulated. He had marked left hemiparesis and this was attributed to edema most likely from XRT. By 5/23 his pupils were fixed bilaterally. He had no doll's eyes response. Corneal reflexes were still present. He had a gag reflex but had no movement of his limbs. At this point, it was decided that he would not receive further XRT or aggressive measures. He subsequently died on 5/24.
