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Case presentation

RLH 03/22

James Lane

Patient

  • 38 Y/O
  • Transferred 02/21 from HUH
  • Presentation
  • 17:00-03:00 at work
  • Sudden onset expressive dysphasia
  • Worsened over shift
  • Mild RSW
  • Went to bed, on waking in am symptoms persistent/worse

NIHSS

NIHSS

0

0

0

0

0

2

0

1

0

0

1 (RUL)

1 (RUL)

2

1

0

- Consciousness

- Questions

- Commands

- Occular movements

- Visual fields

- Facial palsy

- LUL

- RUL

- LLL

- RLL

- Ataxia

- Sensation

- Dysphasia

- Dysarthria

- Neglect

Total = 9

History

  • PMHx
  • Depression
  • Biliary colic
  • Miscarriage x2

  • FMHx
  • Stroke

- father (in 40s)

- brother (aged 50)

  • T2DM

- mother

  • Miscarriage

- mother had 10x

Ix

Management

  • CTH: 2x subacute infarcts L MCA territory
  • CTA: Severe L M1 stenosis
  • Outside tPA window
  • Aspirin 300mg

  • Echo NAD
  • For bubble echo
  • Thrombophilia screen

  • DDx

?Inherited thrombophilia - miscarriage

?haemoglobinopathy

?moyamoya

  • Discharged home
  • Discussed at MDT - refer to Dr Haddadi

MRI

MRI

MRI - "confirms L MCA infarct involving L fronto-parietal regions and extending into deep border zone territory"

CTA - "severe stenosis L M1 cause unclear. Partially occlusive thrombus atherosclerotic stenosis, vasculitis or drug related vasospasm should all be considered

Patient

  • 4 presentations in total
  • 3 positive for acute stroke
  • Young stroke screen negative

  • ? what is causing repeated strokes in a young individual

Further events

Patient

1

  • Re-transferred 06/21
  • Presentation
  • Woke with headache
  • Dysarthria
  • Worsening RSW - face, arm, leg
  • NIHSS 4 (face, RUL/RLL drift, dysarthria)
  • CTH/CTA Nil acute, ongoing L M1 stenosis
  • MRI Nil acute
  • Discharged as decompensation of previous stroke

Patient

2

  • Further admission 11/21
  • Presentation
  • RSW
  • facial droop
  • dysarthria
  • NIHSS 4 (face, RUL/RLL drift, dysarthria)
  • Family Hx - brother now moyamoya

  • MRI - new infarcts L precentral gyrus infarct and R deep white matter

  • Management:
  • DAPT 3/52
  • Clopidogrel monotherapy
  • Avoid aggressive reduction in BP

Patient

  • Further admission 01/22
  • Presentation
  • RSW
  • Facial droop

  • MRI - "Acute L anterior borderzone and R ACA infarcts in the context of new/worsening L ICA, R ICA stenosis"

3

1, 2, 3

Moyamoya

  • Rare condition affecting cerebrovascular blood vessels
  • Stenosis
  • Progressive
  • "puff of smoke"

  • Multiple strokes/TIA - often haemorrhagic

1, 2, 3

Epidemiology

Who?

  • East Asia > Europe/North America (0.35-0.94 per 100000)
  • Incidence
  • Children & adults
  • 2 peaks
  • Female > male (1.9)

1, 2, 3

Aetiology

Why

  • Cause unknown
  • ?Genetic component (?brother)
  • High incidence in Japanese population
  • Familial occurrence 10%-15%
  • R179 in ACTA2, RNF213
  • Secondary
  • Associated with many other conditions

1, 2, 3

Diagnosis

How?

  • Neurovascular imaging

(proposed criteria)

  • Stenosis/occlusion terminal ICA
  • Stenosis/occlusion proximal ACA & MCA
  • Abnormal vascular networks
  • Angiographic findings bilaterally

1, 2, 3

Treatment

2+

  • Symptomatic
  • Reducing ICP
  • Improving cerebral blood flow
  • Managing seizres
  • Neurosurgery for ICH
  • Avoid
  • Hypotension
  • Hyperthermia
  • Hypocarbia
  • Hypovolaemia
  • Treat underlying cause (secondary)
  • Without treatment - multiple strokes

  • Secondary prevention
  • Medical

Antiplatelet

AED if seizures

Anticoagulants if unstable symptoms (rarely long term due to bleeding risk) but avoid

Ca blocker for headache - consider risk of BP reduction

  • Surgical

Revascularisation - stenting/bypass (children>adults)

Pial synangiosis, encephalomyosynangiosis (EMS), encephaloduroarteriosynangiosis (EDAS)

dural inversion.

Summary & Discussion

  • Case to demonstrate Moyamoya and its presentation
  • Considered demographics
  • Considered management

Questions?

References:

1. UpToDate (https://www.uptodate.com/contents/moyamoya-disease-etiology-clinical-features-and-diagnosis?search=moyamoya&source=search_result&selectedTitle=1~46&usage_type=default&display_rank=1)

2. NINDS (https://www.ninds.nih.gov/Disorders/All-Disorders/Moyamoya-Disease-Information-Page#:~:text=Moyamoya%20disease%20is%20a%20rare,to%20compensate%20for%20the%20blockage.

3. Rarediseases.org (https://rarediseases.org/rare-diseases/moyamoya-disease/)

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