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A case of unsteadiness and limb weakness

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A Interesting Case Report

A Interesting Case Report

Published in: Health & Medicine

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  • 1. A Case of Unsteadiness and Limb Weakness Dr Richard McCrory CT2 Medicine 9 th March 2011
  • 2. Case Presentation: Mrs E.W. (1)
    • Previously fit and well 73 year old lady
      • Right Handed
      • Independently Mobile
    • Admitted from A&E Mater Hospital 27/8/2010
    • PC: 1 week history of dizziness, “clumsiness” (especially with the left hand) and unsteadiness on her feet.
    • Referral to A&E prompted by two falls in preceding 24 hours when trying to walk
  • 3. Mrs E.W. (2)
    • PMHx:
    • Hypertension (on Ramipril 2.5mg)
    • Non-smoker, No alcohol
    • Elevated cholesterol (on Simvastatin 40mg)
    • No recent head injury / trauma
    • No family history of neurological disorders
    • Systematic Enquiry:
    • Collateral history suggested episodic forgetfulness past 2 months, occasionally withdrawn and dropped crockery at home.
  • 4. Initial assessment 27/8/10
    • CVS: Pulse 80 regular, BP 139/74 2HS no murmurs, no bruits, no postural BP drop
    • RS: Chest clear
    • Abdomen: NAD
    • Neurological Exam
      • Dysarthric, slight loss nasolabial fold on left
      • Pronator drift left arm, hypotonia
      • Power LUL 4+/5 LLL4+/5, mild truncal ataxia
      • Impaired co-ordination on left side
      • No visual field defect, coarse nystagmus on leftward gaze
      • Abbreviated Mental Test - 8/10
  • 5. Initial Investigations
    • ECG – Normal Sinus Rhythm
    • Bloods – All within normal range, ESR normal, TFT’s normal
    • CXR – Heart size normal, no lung field abnormalities
    • CT Brain (29/08/10)
    • Chronic Ischaemic Periventicular and deep white matter changes, no acute infarct seen. No bleed visible.
  • 6.
    • Initial Clinical Diagnosis - Left Cerebellar Stroke
    • Aspirin 300mg for 2/52
    • MRI Brain + Angiogram booked for assessment of posterior circulation
    • Trans-thoracic Echocardiogram – Normal structure and function, Normal Valves
    • Seen by PT/OT
    • Berg Score 30/8/10 – 32/56
    • Limited safety awareness, mobilised with assistance of 1 + ZF
  • 7. But there’s more…
    • Week 2 of admission:
    • Limited progress with Physio/OT
    • Unsteady on feet
    • Apathetic / Withdrawn – started on SSRI
    • Safety awareness problematic, several IR1 forms re. falls at bedside, poor retention of information, tended to move unsupervised. MMSE 21/30
  • 8.
    • Week 3
    • Choking intermittently on food
      • SLT recommended pureed diet
    • Worsening dysarthria
    • Power LUL 3/5 LLL 4/5
    • Further ischaemia queried – switched to clopidogrel
    • Week 4
    • Progressing truncal and neck ataxia
    • Deteriorating sitting balance, standing assistance of 2
    • Fell out of chair 21/9/10 attempting to stand despite repeated assertions to not mobilise independently
    • Sustained contusion and laceration to right scalp – no loss of consciousness
    • Repeat Berg Score - 4/56
  • 9.
    • 21/9/10
    • Repeat CT Brain – no interval change
    • 30/9/10
    • MRI Brain and Angiogram
      • Bilateral Periventricular Ischaemia
      • Several high signal changes in cerebellar peduncles and left medulla on T2 images
      • ‘ Unusual distribution’ but could correlate with ischaemic changes
      • No vessel abnormalities
      • Started on LMWH for posterior circulation ischaemia
  • 10. But there’s (still) more…
    • Week 5
    • Only safe in bed
    • Unintelligible speech
    • Doubly incontinent
    • Evolving right sided cerebellar signs
    • Deteriorating swallow – referred to dietician for NG tube and enteral feeds, and on IV fluids
    • Repeat bloods – no signs of infection / inflammation
    • Re-evaluated initial diagnosis and differential, proceeded to Lumbar Puncture
  • 11. Investigations
    • Lumbar Puncture (09/10/10)
    • Clear colourless fluid
    • CSF glucose – Normal
    • Gram Stain and Culture – Negative
    • Cell Count – WCC 5 cells/mm3
    • CSF Protein elevated: 0.79g/dl (Normal range 0.1 – 0.3 g/dl), confirmed on repeat LP
    • ANCA/ANA/Serum ACE / Oligolonal Bands – Negative
    • HSV/CMV PCR on CSF - Negative
  • 12.
    • Sought Neurology advice from RVH
    • Advised
    • Check Anti-Neuronal Antibodies – sent to London
    • Breast Exam - Normal
    • CT Chest / Abdomen / Pelvis to seek occult malignancy
  • 13. CT Chest/Abdo/Pelvis 19/10/10
    • No evidence of mediastinal or para-aortic lymphadenopathy
    • Lung fields and visceral organs appeared normal
    • However a 2.5 x 1.7 cm soft tissue mass was identified in the right breast.
      • Plans made for transfer to BCH breast clinic for triple assessment
  • 14.
    • Became unwell with Tachycardia, Tachypnoea
    • CXR noted new pulmonary filling defects consistent with consolidation
    • Started Tazocin
    • Blood cultures positive for Methicillin Sensitive Staph Aureus
    • Possible venflon associated infection
    • Switched to Vancomycin / Meropenem
  • 15. Final Diagnosis – Paraneoplastic Cerebellar Degeneration secondary to Primary B-Cell Lymphoma of the Breast
  • 16. The Cerebellum – A Brief Overview of Functional Anatomy
    • Archicerebellum – maintenance of equilibrium
    • Paleocerebellum – muscle tone and posture
    • Neocerebellum – muscular co-ordination
  • 17. Paraneoplastic Neurological Syndromes
    • ‘ A humoral or immune-mediated mechanism other than a metastatic complication in patients with an underlying malignancy.’
    • ‘ Remote effect’ immune mediated CNS pathology affects 1-3% of all cancer patients.
  • 18. Paraneoplastic cerebellar degeneration (PCD)
    • Constitutes 25-35% of paraneoplastic neurologic syndromes diagnosed.
    • Characterised by diffuse loss of Purkinje cells throughout the cerebellar cortex.
    • Antibodies directed to Purkinje cytoplasmic and nuclear proteins regulating cell survival trigger apoptosis
      • Anti-Yo, Anti-Tr, Anti-Hu plus others
      • 40% no recognisable antibody identified
  • 19.
    • In 60–70% of patients, neurological symptoms precede diagnosis of the cancer by a few months to 2–3 years.
    • Common Neoplasms associated with PCD
      • Breast and Ovary (Anti-Yo)
      • Small Cell Lung Cancer (Anti-Hu, Anti-Ri)
      • Lymphoma (Anti-Tr highly specific)
  • 20. Clinical Features of PCD
    • Mild unilateral cerebellar signs evolving (days-weeks) into severe bilateral cerebellar dysfunction, then symptoms stabilise with profound physical disability.
      • Mild cognitive deficits as well as affective symptoms seen in 20% of cases (Cerebellar Cognitive Affective Syndrome)
  • 21. A Large Diagnostic Differential
    • Cerebrovascular
      • Ischaemic or Haemorrhagic Stroke
    • Toxins
      • Alcohol / Chemotherapy / Anticonvulsants
    • Inflammatory Disorders
      • Multiple Sclerosis / Neurosarcoidosis
    • Encephalomyelitis
    • Intracranial Neoplasm
      • Primary CNS / Metastatic / Leptomeningeal
    • Neurodegenerative Disorders
      • Spinocerebellar Ataxia (Sporadic)
      • Prion Related Diseases
  • 22. Findings not Consistent with paraneoplastic cerebellar degeneration
    • Include the following:
    • Severely altered mental status with myoclonus and ataxia
    • Predominantly corticospinal tract dysfunction
    • Unilateral cerebellar dysfunction
    • Familial cerebellar degeneration
  • 23. Investigations
    • CT / MRI Brain may be initially normal
      • Cerebellar atrophy more pronounced in latter stages of disease
    • Lumbar Puncture
      • High CSF protein, Pleocytosis
      • Can identify auto-antibodies in CSF and help exclude leptomeningeal disease
    • CT / PET to look for occult malignancy
  • 24. Treatment
    • Variable but generally unsatisfactory
      • Complete and Partial remission possible but uncommon
    • Approach 1
    • Remove antigen source (Tumour)
      • Surgery, Chemoradiotherapy as applicable
    • Approach 2
    • Suppress immune response
      • Steroids, Cyclophospamide, Rituximab
  • 25. Prognosis
    • Commonly disability correlates with onset of treatment – ‘The Horse has bolted’
    • May require extended follow-up if occult malignancy suspected
    • Oncologic outcome of patients with antibody-associated paraneoplastic syndromes does not significantly differ from that of patients without syndrome.
  • 26. Take Home Messages
    • Consider a diagnosis of PCD in patients who present with acute or subacute cerebellar degeneration and no risk factors for cerebellar disorders
    • Identification of specific auto-antibodies may help guide diagnostic assessment
  • 27.