Case presentation Omar Salim ST5 Neurology o.salim@nhs.net June - - PowerPoint PPT Presentation

Case presentation

Omar Salim ST5 Neurology

• .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.
• 2nd 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

3rd (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

7th 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 risingà 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!