A Case of Oro-Facio-Bulbar weakness

2,169 views

Published on

Published in: Health & Medicine
0 Comments
0 Likes
Statistics
Notes
  • Be the first to comment

  • Be the first to like this

No Downloads
Views
Total views
2,169
On SlideShare
0
From Embeds
0
Number of Embeds
16
Actions
Shares
0
Downloads
19
Comments
0
Likes
0
Embeds 0
No embeds

No notes for slide

A Case of Oro-Facio-Bulbar weakness

  1. 1. DR.MAHESH KUMAR’S UNIT.
  2. 2. Case history <ul><li>Sikkandar ,70 yrs male,retd from TNEB </li></ul><ul><li>3 months ago,initially had fever /URI/ear block which lasted for 5 days,subsided with RX. </li></ul><ul><li>Later noticed insidious onset of diplopia&difficulty in eye closure  nasal speech  difficulty in chewing/swallowing&nasal regurgitation. </li></ul><ul><li>No h/o </li></ul><ul><li>difficulty in smell perception. </li></ul><ul><li>disturbances in color vision. </li></ul><ul><li>altered sensory perception over face . </li></ul>
  3. 3. <ul><li>No h/o </li></ul><ul><li>HOH/tinnitus/vertigo </li></ul><ul><li>difficulty in turning from side to side/shoulder shrug </li></ul><ul><li>motor weakness of the arms & legs </li></ul><ul><li>sensory deficit/positive/negative sensory phenomena </li></ul><ul><li>involuntary movements </li></ul><ul><li>seizures/headache/vomiting/head injury </li></ul><ul><li>previous similar episodes </li></ul><ul><li>Not a known TB/DM/HT patient;no high risk behaviour. </li></ul>
  4. 4. <ul><li>o/e </li></ul><ul><li>no neurocutaneous markers </li></ul><ul><li>ht neck ratio normal </li></ul><ul><li>vitals stable. </li></ul><ul><li>Examination of CNS: </li></ul><ul><li>HF:normal,MMSE:28/30 </li></ul>
  5. 5. Cranial nerves Rt Lt 1.Smell perception Normal Normal 2.Visual acuity field of vision color vision fundus Normal Normal 3,4,6 palpebral fissure pupil size&reacn EOM ptosis + 3 mm,reacn normal Full Ptosis+ 3 mm ,reacn normal Full 5.Sensory perception muscles of mastication jaw jerk Normal Weak Not exaggerated Normal Weak Not exaggerated 7.Raising eyebrows eye closure pursing &whistling taste over ant 2/3 Weak Not complete Not possible Normal Weak Not complete Not possible Normal
  6. 6. 8. Rinne ‘ s test weber ‘s test Positive Not lateralized Positive Not lateralized 9&10Palatal reflex gag reflex diminished diminished 11. Power of SCM shoulder shrug Normal Normal 12. Size wasting strength fasciculations Normal No Decreased No Normal No Decreased No
  7. 7. <ul><li>Motor system: </li></ul><ul><li>no muscle wasting/weakness </li></ul><ul><li>supercial &deep tendon reflexes normal </li></ul><ul><li>Gait normal </li></ul><ul><li>Sensory system:normal </li></ul><ul><li>No cerebellar signs </li></ul><ul><li>Spine &cranium normal </li></ul><ul><li>Other systems:no abnormality detected. </li></ul>
  8. 8. <ul><li>orofaciobulbar weakness </li></ul><ul><li>DD? </li></ul>
  9. 9. DD of orofaciobulbar weakness <ul><li>1.Neurasthenia /depression </li></ul><ul><li>2.Progressive external ophthalmoplegia </li></ul><ul><li>3.Polymyositis /inclusion body myositis </li></ul><ul><li>4.Congenital myasthenic states </li></ul><ul><li>5.Progressive bulbar palsy </li></ul><ul><li>6.Multiple sclerosis </li></ul><ul><li>7.Stroke </li></ul><ul><li>8.GBS variants –Miller-fisher variant </li></ul><ul><li>9.Initial stages of botulism </li></ul>
  10. 10. DD -contd.. <ul><li>MCNP syndromes: </li></ul><ul><li>Intracranial –extramedullary or extracranial processes </li></ul><ul><li>1.Neoplastic meningitis </li></ul><ul><li>2.Nasopharyngeal carcinoma </li></ul><ul><li>3.Osteopetrosis </li></ul><ul><li>4.Vertebro-basilar dolichoectasia </li></ul><ul><li>5.Neurosarcoidosis </li></ul><ul><li>6.Polyneuritis cranialis(GBS variant) </li></ul><ul><li>7.Bannwarth ‘s syndrome(lyme disease) </li></ul>
  11. 11. Investigations done <ul><li>CBC:Hb :11 g% TC:8000 DC:P65L33E2 ESR:2/5 </li></ul><ul><li>RFT: Sugar:110 urea:22 creatinine:0.7 </li></ul><ul><li>ECG:NSR,WNL </li></ul><ul><li>CXR:WNL </li></ul><ul><li>MRI BRAIN:No significant abnormality </li></ul><ul><li>RNS:Done at 2 HZ,recording from the orbicularis oculi,nasalis,deltoid. </li></ul><ul><li>Normal amplitudes obtained with significant decremental response in the nasalis,deltoid&orbicularis;consistent with MG.s </li></ul>
  12. 12. <ul><li>Sr AchR abs:18.98(neg <0.25;positive>0.40) </li></ul><ul><li>CT thorax:No significant abnormality </li></ul><ul><li>TFT:normal </li></ul><ul><li>Rhematoid factor:negative </li></ul><ul><li>CRP:negative </li></ul><ul><li>ANA:1:10 dilution &1:40 dilution positive;speckled </li></ul><ul><li>ECHO:normal Lv systolic function;no RWMA </li></ul>
  13. 13. Treatment given <ul><li>Started on , </li></ul><ul><li>T.Pyridostigmine 60 mg qid </li></ul><ul><li>T.Prednisolone 5 mg 2 od </li></ul>
  14. 14. <ul><li>MYASTHENIA GRAVIS </li></ul>
  15. 15. Myasthenia gravis <ul><li>A neuromuscular disorder, </li></ul><ul><li>Characterised by, </li></ul><ul><li>1.weakness &fatiguability of skeletal muscles </li></ul><ul><li>2.decrease in no of AchR at the NMJ due to an </li></ul><ul><li>antibody mediated autoimmune attack. </li></ul>
  16. 17. Pathophysiology <ul><li>Decrease in the no of AchR at the post-synaptic membrane;flattening of post-synaptic folds. </li></ul><ul><li>Even with normal release of Ach end-plate potentials are small  failure to trigger MAP. </li></ul><ul><li>Neuromuscular abnormalities d/t AchR abs. </li></ul><ul><li>The abs are IgG and T cell dependent. </li></ul><ul><li>The thymus plays a role in this process. </li></ul><ul><li>?Myoid cells with AchR on surface-autoantigen </li></ul>
  17. 18. Clinical features <ul><li>All age groups, women in 20-40 yrs&men in 50-60 yrs. </li></ul><ul><li>Weakness increases with repeated use,may improve following rest. </li></ul><ul><li>Course variable with remissions and exacerbations. </li></ul><ul><li>Remissions rarely complete. </li></ul>
  18. 19. Muscle weakness-distribution <ul><li>Cranial muscles: </li></ul><ul><li>1.lids &EOM are often the first affected. </li></ul><ul><li>2.facial weakness/ weakness in chewing. </li></ul><ul><li>3.nasal timbre to speech(palate)/dysarthric(tongue) </li></ul><ul><li>4.difficulty in swallowing/regurgitation. </li></ul><ul><li>5.bulbar weakness-esp with anti MUSK ab </li></ul><ul><li>Limb muscles </li></ul><ul><li>1.weakness generalizes in 80% </li></ul><ul><li>2.often proximal and asymmetric. </li></ul>
  19. 20. <ul><li>Others </li></ul><ul><li>axial muscles. </li></ul><ul><li>diaphragm/abdominal ms/intercostals. </li></ul><ul><li>even the external sphincter of bladder&bowel. </li></ul><ul><li>Preserved DTR despite muscle weakness </li></ul>
  20. 21. Osserman’s grading Grade Weakness Progress Crises Drug response Incidence I Ocular ? No satisfactory 15-20% II A Mild generalized slow No satisfactory 30% II B Moderately Severe generalized Slow No Less than satisfactory 25% III Acute Fulminant Rapid yes Poor 15% IV Late severe Steady Progression over 2 yrs yes poor 10%
  21. 22. Diagnosis <ul><li>history/physical examination </li></ul><ul><li>Lab </li></ul><ul><li>1.anti AchR radioimmunoassay </li></ul><ul><li>85% positive in generalized MG,50% in ocular </li></ul><ul><li>MG </li></ul><ul><li>40% of negative pts have antiMUSK abs. </li></ul><ul><li>2.repetitive nerve stimulation </li></ul><ul><li>3.single fiber EMG </li></ul><ul><li>4.tensilon test </li></ul>
  22. 23. Repetitive nerve stimulation <ul><li>AchEmedication stopped 6-24 hrs before </li></ul><ul><li>Best to test weak/proximal muscles </li></ul><ul><li>Repetitive stimulation of the nerve at 3/sec </li></ul><ul><li>Decremental response (decrease in muscle CMAP)of atleast 10-15% </li></ul><ul><li>Edrophonium can prevent this response. </li></ul>
  23. 24. Single fiber EMG <ul><li>More sensitive than RNS. </li></ul><ul><li>Identification of APs from single muscle fibers </li></ul><ul><li>Inconstancy of the normally invariant interval between firing of fibers connected to same motor unit(jitter)/blocking of successive discharges. </li></ul><ul><li>NCV &distal latencies are normal </li></ul>
  24. 25. Tensilon (AchE)test <ul><li>Reserved for pts with neg abs/EDS. </li></ul><ul><li>Edrophonium;onset:30 s,DOA:5 min </li></ul><ul><li>An objective endpoint selected </li></ul><ul><li>Given in two divided doses(2+8 mg) to avoid sideeffect </li></ul><ul><li>Atropine should be kept ready. </li></ul><ul><li>False +: ALS,placebo reactors </li></ul>
  25. 26. Disorders a/w MG <ul><li>1.Thymus </li></ul><ul><li>Thymoma,hyperplasia </li></ul><ul><li>2.Thyroid </li></ul><ul><li>3.Autoimmune </li></ul><ul><li>RA,SLE,sjogren’s and others </li></ul><ul><li>4.Exacerbation of MG </li></ul><ul><li>Hypo/hyperthyroidism,occult infn,stress etc </li></ul><ul><li>5.Interference with therapy </li></ul><ul><li>TB,DM,GIB,HT,BA,osteoporosis,obesity etc </li></ul>
  26. 27. lab tests <ul><li>CT/MRI of mediastinum </li></ul><ul><li>ANA/RF/anti thyroid abs </li></ul><ul><li>PPD skin test </li></ul><ul><li>CXR </li></ul><ul><li>FBS/HbA1c </li></ul><ul><li>PFT </li></ul><ul><li>Bone densitometry in older pts. </li></ul>
  27. 28. Treatment <ul><li>Anticholinesterase drugs </li></ul><ul><li>Thymectomy </li></ul><ul><li>Immunosuppressive agents </li></ul><ul><li>Plasmapheresis &IVIg </li></ul>
  28. 30. <ul><li>Pyridostigmine most widely used. </li></ul><ul><li>Action begins in 15-30 min,lasts for 3-4 hrs. </li></ul><ul><li>Rx started with 30-60 mg tds to qid. </li></ul><ul><li>Tailored to individual requirements. </li></ul><ul><li>Max useful dose rarely exceeds 120 mg every 3-6 hr. </li></ul><ul><li>Over dosage may increased weakness. </li></ul><ul><li>Muscarinic side effects in a few. </li></ul><ul><li>Atropine/diphenoxylate can be used. </li></ul>
  29. 31. Thymectomy <ul><li>Advantages : </li></ul><ul><li>85% experience remission,drug-free remission in 35% </li></ul><ul><li>Improvement typically delayed for months to yrs. </li></ul><ul><li>Definite: </li></ul><ul><li>All pts between puberty&55 yrs. </li></ul><ul><li>Those with thymoma. </li></ul><ul><li>Doubtful : </li></ul><ul><li>Children & those >55 yrs. </li></ul><ul><li>Ocular MG,MUSK ab positivity. </li></ul>
  30. 32. Immunosuppression <ul><li>Immediate improvement : </li></ul><ul><li>IVIg </li></ul><ul><li>Plasmapheresis </li></ul><ul><li>Intermediate term: 1-3 months </li></ul><ul><li>Glucocorticoids </li></ul><ul><li>Cyclosporine </li></ul><ul><li>Tacrolimus </li></ul><ul><li>Long term: </li></ul><ul><li>Mycophenolate mofetil /Azathioprine </li></ul>
  31. 33. <ul><li>Glucocorticoid therapy: </li></ul><ul><li>Given in a single dose. </li></ul><ul><li>Low initial dose(15-25 mg/d),increased stepwise. </li></ul><ul><li>Until marked improvement/50-60 mg/d reached. </li></ul><ul><li>Gradually modified to an alternate day regimen. </li></ul><ul><li>Most common errors with steroid RX in MG: </li></ul><ul><li>1.Insufficient persistence </li></ul><ul><li>2.Too early/rapid/excessive dose tapering. </li></ul><ul><li>3. Lack of attention to side effects. </li></ul>
  32. 34. Myasthenic crisis <ul><li>Exacerbation of weakness usually with respiratory failure caused by diaphragm&intercostal muscle weakness. </li></ul><ul><li>Rarely occurs in properly managed persons. </li></ul><ul><li>Anticholinesterases temporarily stopped. </li></ul><ul><li>RX:antibiotics ,supportive measures </li></ul><ul><li>Plasmapheresis:usually 5 exchanges over a 10-14 day period. </li></ul><ul><li>IVIg:usually 2 g/kg given over 5 days </li></ul><ul><li>Both have intermittent benefit& are costly </li></ul>
  33. 35. Immunosuppresive drugs <ul><li>Mycophenolate mofetil: </li></ul><ul><li>1-1.5 g bd </li></ul><ul><li>relative lack of side effects,high cost </li></ul><ul><li>Azathioprine : </li></ul><ul><li>2-3 mg/kg ,beneficial effect takes 3 -6 months to begin </li></ul><ul><li>Should never be given Allopurinol </li></ul><ul><li>Cyclosporine/Tacrolimus: </li></ul><ul><li>4-5mg/kg &0.1 mg/kg/d resply;nephrotoxic </li></ul><ul><li>Cyclophosphamide :reserved for refractory cases </li></ul>
  34. 36. DRUGS & MG <ul><li>Drugs that may exacerbate MG </li></ul><ul><li>Antibiotics :aminoglycosides,quinolones,macrolides </li></ul><ul><li>Nondepolarising muscle relaxants(curare) </li></ul><ul><li>Beta blockers </li></ul><ul><li>Local anaesthetics &related agents </li></ul><ul><li>Quinine derivatives </li></ul><ul><li>Magnesium </li></ul><ul><li>Penicillamine </li></ul><ul><li>Botulinum toxins </li></ul>
  35. 37. <ul><li>THANK YOU </li></ul>

×