Case presentation Omar Salim ST5 Neurology o.salim@nhs.net
June 2010 • 32M, Rt-handed, Van driver • admitted with 2/52 of blurred vision in the right eye followed by gradual onset of weakness in the left arm and leg, together with some cognitive and memory disturbance. • Unremarkable PMH or FHx • Smoker, no alcohol
Examination • GCS 14 (confused) • Speech normal. • VA CF on right and 6/9 on the left. • No papilloedema. • left homonymous hemianopia. • Eye movements normal. No facial weakness • Left pyramidal weakness. • plantars extensor bilaterally. • MRI scan showed …..
MRI report • Multifocal lesions with increased T2 signal in the right occipital lobe spread across the splenium of the CC to the left. • A further discrete area of high signal in the left occipital lobe and a moderate sized area of high signal in the right frontal lobe and another in the right parietal lobe. • Patchy enhancement of the large right occipital and corpus callosal lesion. • No major mass effect. • Chest x-ray and CT scan of the thorax, abdomen and pelvis with contrast were unremarkable. • What else do you want at this stage?
Ongoing Investigations…. • brain biopsy in June revealed necrotic tissue with no evidence of either inflammation or neoplasia. • Admission complicated by Massive PE and DVT of left femoral vein. • Clinically deteriorated, transferred to ICU. 2 nd brain biopsy in July showed few reactive astrocytes, felt changes likely to be reactive or inflammatory rather than • neoplastic, but biopsy was considered non-diagnostic! • CSF : Acellular, Glu of 5.2, Ptn insufficient. No organisms. CSF toxoplasma serology -ve.. OCBs negative . • Bone marrow biopsy: no evidence of lymphoma. • Another CSF 4/52 later: WBC 0, RBC 1157, protein 0.27 g/L, glucose 4.2 (4.7). CSF Tropheryma whippelii PCR was negative. OCBs were also negative . AFB and subsequent CSF TB culture were negative. • Early September, complained of worsening headaches. An MRI scan showed haemorrhagic change within posterior part of the right occipital lesion
3 rd (and final) brain biopsy in September ‘ Reactive astrocytes, perivascular cuffing by lipophages with absence of myelin. A few scattered T-cells were seen. The conclusion was that, there was a demyelinating process of recent onset and the appearances were compatible with MS ’ • Received 5/7 IVMP followed by oral Prednisolone 80 mg. • Over subsequent weeks, there was some improvement in his cognitive state, he remained with a severe left hemiparesis and left homonymous hemianopia.
Which DMT? • October, received his first infusion of Tysabri. • The dose of Prednisolone was gradually reduced. • Follow up MRI scans of the brain in November and December showed some improvement in the size of the cerebral lesions .
May 2011 • Frequent left-sided partial motor seizures • Will you hold Tysabri? • Would you repeat his CSF for JCV? • Subsequent CSF JC virus testing was negative. • Started on Lamotrigine. Still on Prednisolone 25 mg • Neurologically no change, able to stand and transfer with the help of one person. EDSS 7
Nov 2017 • JCV Ab titre index came back +ve at 1.55 • What will you do? • ?CSF for JCV • 3 months wash out period • Next DMT?...
June 2018 • MR scan stable • No relapses or new symptoms since starting Fingolimod, in fact felt much better on it and even his mood has improved. • EDSS remains 7.5
April 2019 • Admitted to a local DGH with 3 days of dysphagia and dysarthria • CT scan in ED showed nil acute • Discharged home!
• Readmitted 2 days later with worsening symptoms and new left sided weakness • MRI..
April 2019 7 th April at DGH
Over next few days.. • Started on IVMP • Clinically deteriorated.. Progressive dysphagia (NGT) and dysarthria • Progressive Rt sided weakness • GCS dropped to 10 (E3, M5, V2) • Breathing difficulty, NEWS score rising à T1RF then within 2 hours moved into T2RF • Transferred to ITU, I&V
Next Battle! • IVMP extended for another 5/7 (total 8/7) à Pred 60 mg • PLEX started… • CSF acellular, ptn 0.65, Glu normal, JCV pending • Battle with ITU! • HAP, Trache • Next?? • Cyclophosphamide pulse given • EEG Rt cortical dysfunction, no epilept
Few days ago • Stepped down to Neurology ward • GCS still ~10 (M6, V1, E3) • Plan to have full 6* cycles of Cyclophosphamide
Thank you!
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