A Case of Unsteadiness and Limb Weakness Dr Richard McCrory CT2 Medicine 9 th  March 2011
Case Presentation: Mrs E.W. (1) <ul><li>Previously fit and well 73 year old lady </li></ul><ul><ul><li>Right Handed </li><...
Mrs E.W. (2) <ul><li>PMHx: </li></ul><ul><li>Hypertension (on Ramipril 2.5mg) </li></ul><ul><li>Non-smoker, No alcohol </l...
Initial assessment 27/8/10 <ul><li>CVS:  Pulse 80 regular, BP 139/74 2HS no murmurs, no bruits, no postural BP drop </li><...
Initial Investigations <ul><li>ECG  – Normal Sinus Rhythm </li></ul><ul><li>Bloods  – All within normal range, ESR normal,...
<ul><li>Initial Clinical Diagnosis -  Left Cerebellar Stroke </li></ul><ul><li>Aspirin 300mg for 2/52 </li></ul><ul><li>MR...
But there’s more… <ul><li>Week 2 of admission: </li></ul><ul><li>Limited progress with Physio/OT </li></ul><ul><li>Unstead...
<ul><li>Week 3 </li></ul><ul><li>Choking intermittently on food </li></ul><ul><ul><li>SLT recommended pureed diet </li></u...
<ul><li>21/9/10  </li></ul><ul><li>Repeat CT Brain  – no interval change </li></ul><ul><li>30/9/10 </li></ul><ul><li>MRI B...
But there’s (still) more… <ul><li>Week 5 </li></ul><ul><li>Only safe in bed </li></ul><ul><li>Unintelligible speech </li><...
Investigations <ul><li>Lumbar Puncture (09/10/10) </li></ul><ul><li>Clear colourless fluid </li></ul><ul><li>CSF glucose –...
<ul><li>Sought Neurology advice from RVH </li></ul><ul><li>Advised </li></ul><ul><li>Check  Anti-Neuronal Antibodies  – se...
CT Chest/Abdo/Pelvis 19/10/10 <ul><li>No evidence of mediastinal or para-aortic lymphadenopathy </li></ul><ul><li>Lung fie...
<ul><li>Became unwell with Tachycardia, Tachypnoea </li></ul><ul><li>CXR noted new pulmonary filling defects consistent wi...
Final Diagnosis –  Paraneoplastic Cerebellar Degeneration secondary to Primary B-Cell Lymphoma of the Breast
The Cerebellum – A Brief Overview of Functional Anatomy <ul><li>Archicerebellum – maintenance of equilibrium </li></ul><ul...
Paraneoplastic Neurological Syndromes <ul><li>‘ A humoral or immune-mediated mechanism other than a metastatic complicatio...
Paraneoplastic cerebellar degeneration (PCD) <ul><li>Constitutes 25-35% of paraneoplastic neurologic syndromes diagnosed. ...
<ul><li>In 60–70% of patients, neurological symptoms precede diagnosis of the cancer by a few months to 2–3 years. </li></...
Clinical Features of PCD <ul><li>Mild unilateral cerebellar signs evolving (days-weeks) into severe bilateral cerebellar d...
A Large Diagnostic Differential <ul><li>Cerebrovascular </li></ul><ul><ul><li>Ischaemic or Haemorrhagic Stroke </li></ul><...
Findings  not  Consistent with   paraneoplastic cerebellar degeneration  <ul><li>Include the following: </li></ul><ul><li>...
Investigations <ul><li>CT / MRI Brain may be initially normal </li></ul><ul><ul><li>Cerebellar atrophy more pronounced in ...
Treatment <ul><li>Variable but generally unsatisfactory </li></ul><ul><ul><li>Complete and Partial remission possible but ...
Prognosis <ul><li>Commonly disability correlates with onset of treatment – ‘The Horse has bolted’ </li></ul><ul><li>May re...
Take Home Messages <ul><li>Consider a diagnosis of PCD in patients who present with acute or subacute cerebellar degenerat...
 
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A case of unsteadiness and limb weakness

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A case of unsteadiness and limb weakness

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