A Case of Oro-Facio-Bulbar weakness
-
Upload
stanley-medical-college-department-of-medicine -
Category
Health & Medicine
-
view
2.171 -
download
8
description
Transcript of A Case of Oro-Facio-Bulbar weakness
![Page 1: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/1.jpg)
DR.MAHESH KUMAR’S UNIT.
![Page 2: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/2.jpg)
Case historySikkandar ,70 yrs male,retd from TNEB3 months ago,initially had fever /URI/ear block
which lasted for 5 days,subsided with RX.Later noticed insidious onset of diplopia&difficulty
in eye closurenasal speechdifficulty in chewing/swallowing&nasal regurgitation.
No h/o difficulty in smell perception. disturbances in color vision. altered sensory perception over face .
![Page 3: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/3.jpg)
No h/o
HOH/tinnitus/vertigo
difficulty in turning from side to side/shoulder shrug
motor weakness of the arms & legs
sensory deficit/positive/negative sensory phenomena
involuntary movements
seizures/headache/vomiting/head injury
previous similar episodes
Not a known TB/DM/HT patient;no high risk behaviour.
![Page 4: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/4.jpg)
o/e no neurocutaneous markers ht neck ratio normal vitals stable.Examination of CNS:HF:normal,MMSE:28/30
![Page 5: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/5.jpg)
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 normalFull
Ptosis+3 mm ,reacn normalFull
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 completeNot possibleNormal
Weak Not completeNot possibleNormal
![Page 6: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/6.jpg)
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
![Page 7: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/7.jpg)
Motor system: no muscle wasting/weakness supercial &deep tendon reflexes normalGait normalSensory system:normalNo cerebellar signsSpine &cranium normalOther systems:no abnormality detected.
![Page 8: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/8.jpg)
orofaciobulbar weakness
DD?
![Page 9: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/9.jpg)
DD of orofaciobulbar weakness1.Neurasthenia /depression2.Progressive external ophthalmoplegia3.Polymyositis /inclusion body myositis4.Congenital myasthenic states5.Progressive bulbar palsy6.Multiple sclerosis7.Stroke 8.GBS variants –Miller-fisher variant9.Initial stages of botulism
![Page 10: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/10.jpg)
DD -contd..MCNP syndromes: Intracranial –extramedullary or extracranial
processes1.Neoplastic meningitis2.Nasopharyngeal carcinoma3.Osteopetrosis4.Vertebro-basilar dolichoectasia5.Neurosarcoidosis 6.Polyneuritis cranialis(GBS variant)7.Bannwarth ‘s syndrome(lyme disease)
![Page 11: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/11.jpg)
Investigations done CBC:Hb :11 g% TC:8000 DC:P65L33E2 ESR:2/5RFT: Sugar:110 urea:22 creatinine:0.7ECG:NSR,WNLCXR:WNLMRI BRAIN:No significant abnormalityRNS:Done at 2 HZ,recording from the orbicularis
oculi,nasalis,deltoid.Normal amplitudes obtained with significant
decremental response in the nasalis,deltoid&orbicularis;consistent with MG.s
![Page 12: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/12.jpg)
Sr AchR abs:18.98(neg <0.25;positive>0.40)CT thorax:No significant abnormalityTFT:normalRhematoid factor:negativeCRP:negativeANA:1:10 dilution &1:40 dilution
positive;speckledECHO:normal Lv systolic function;no RWMA
![Page 13: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/13.jpg)
Treatment givenStarted on ,T.Pyridostigmine 60 mg qidT.Prednisolone 5 mg 2 od
![Page 14: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/14.jpg)
MYASTHENIA GRAVIS
![Page 15: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/15.jpg)
Myasthenia gravisA neuromuscular disorder,Characterised by, 1.weakness &fatiguability of skeletal
muscles 2.decrease in no of AchR at the NMJ due
to an antibody mediated autoimmune
attack.
![Page 16: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/16.jpg)
![Page 17: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/17.jpg)
PathophysiologyDecrease in the no of AchR at the post-synaptic
membrane;flattening of post-synaptic folds.Even with normal release of Ach end-plate
potentials are smallfailure to trigger MAP.Neuromuscular abnormalities d/t AchR abs.The abs are IgG and T cell dependent.The thymus plays a role in this process.?Myoid cells with AchR on surface-autoantigen
![Page 18: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/18.jpg)
Clinical featuresAll age groups, women in 20-40 yrs&men in
50-60 yrs.Weakness increases with repeated use,may
improve following rest.Course variable with remissions and
exacerbations.Remissions rarely complete.
![Page 19: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/19.jpg)
Muscle weakness-distributionCranial muscles: 1.lids &EOM are often the first affected. 2.facial weakness/ weakness in chewing. 3.nasal timbre to speech(palate)/dysarthric(tongue) 4.difficulty in swallowing/regurgitation. 5.bulbar weakness-esp with anti MUSK abLimb muscles 1.weakness generalizes in 80% 2.often proximal and asymmetric.
![Page 20: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/20.jpg)
Others axial muscles. diaphragm/abdominal ms/intercostals. even the external sphincter of
bladder&bowel.Preserved DTR despite muscle weakness
![Page 21: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/21.jpg)
Osserman’s grading Grade Weaknes
sProgress Crises Drug
responseIncidence
I Ocular ? No satisfactory
15-20%
II A Mild generalized
slow No satisfactory
30%
II B Moderately Severegeneralized
Slow No Less than satisfactory
25%
III Acute Fulminant
Rapid yes Poor 15%
IV Late severe
Steady Progression over 2 yrs
yes poor 10%
![Page 22: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/22.jpg)
Diagnosis history/physical examinationLab 1.anti AchR radioimmunoassay 85% positive in generalized MG,50% in
ocular MG 40% of negative pts have antiMUSK abs. 2.repetitive nerve stimulation 3.single fiber EMG 4.tensilon test
![Page 23: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/23.jpg)
Repetitive nerve stimulationAchEmedication stopped 6-24 hrs beforeBest to test weak/proximal musclesRepetitive stimulation of the nerve at 3/secDecremental response (decrease in muscle
CMAP)of atleast 10-15%Edrophonium can prevent this response.
![Page 24: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/24.jpg)
Single fiber EMGMore sensitive than RNS.Identification of APs from single muscle
fibersInconstancy of the normally invariant interval
between firing of fibers connected to same motor unit(jitter)/blocking of successive discharges.
NCV &distal latencies are normal
![Page 25: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/25.jpg)
Tensilon (AchE)testReserved for pts with neg abs/EDS.Edrophonium;onset:30 s,DOA:5 minAn objective endpoint selectedGiven in two divided doses(2+8 mg) to avoid
sideeffectAtropine should be kept ready.False +: ALS,placebo reactors
![Page 26: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/26.jpg)
Disorders a/w MG1.Thymus Thymoma,hyperplasia2.Thyroid3.Autoimmune RA,SLE,sjogren’s and others4.Exacerbation of MG Hypo/hyperthyroidism,occult infn,stress etc5.Interference with therapy TB,DM,GIB,HT,BA,osteoporosis,obesity etc
![Page 27: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/27.jpg)
lab testsCT/MRI of mediastinumANA/RF/anti thyroid absPPD skin testCXRFBS/HbA1cPFTBone densitometry in older pts.
![Page 28: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/28.jpg)
Treatment Anticholinesterase drugsThymectomyImmunosuppressive agentsPlasmapheresis &IVIg
![Page 29: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/29.jpg)
![Page 30: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/30.jpg)
Pyridostigmine most widely used.Action begins in 15-30 min,lasts for 3-4 hrs.Rx started with 30-60 mg tds to qid.Tailored to individual requirements.Max useful dose rarely exceeds 120 mg every
3-6 hr.Over dosage may increased weakness.Muscarinic side effects in a few.Atropine/diphenoxylate can be used.
![Page 31: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/31.jpg)
ThymectomyAdvantages :85% experience remission,drug-free remission in 35%Improvement typically delayed for months to yrs.Definite: All pts between puberty&55 yrs.Those with thymoma.Doubtful :Children & those >55 yrs.Ocular MG,MUSK ab positivity.
![Page 32: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/32.jpg)
Immunosuppression Immediate improvement : IVIg Plasmapheresis Intermediate term: 1-3 months Glucocorticoids Cyclosporine Tacrolimus Long term: Mycophenolate mofetil /Azathioprine
![Page 33: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/33.jpg)
Glucocorticoid therapy: Given in a single dose. Low initial dose(15-25 mg/d),increased stepwise. Until marked improvement/50-60 mg/d reached. Gradually modified to an alternate day regimen.Most common errors with steroid RX in MG: 1.Insufficient persistence 2.Too early/rapid/excessive dose tapering. 3. Lack of attention to side effects.
![Page 34: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/34.jpg)
Myasthenic crisisExacerbation of weakness usually with respiratory
failure caused by diaphragm&intercostal muscle weakness.
Rarely occurs in properly managed persons.Anticholinesterases temporarily stopped.RX:antibiotics ,supportive measuresPlasmapheresis:usually 5 exchanges over a 10-14
day period.IVIg:usually 2 g/kg given over 5 daysBoth have intermittent benefit& are costly
![Page 35: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/35.jpg)
Immunosuppresive drugsMycophenolate mofetil:1-1.5 g bd relative lack of side effects,high costAzathioprine :2-3 mg/kg ,beneficial effect takes 3 -6 months to
beginShould never be given AllopurinolCyclosporine/Tacrolimus:4-5mg/kg &0.1 mg/kg/d resply;nephrotoxicCyclophosphamide :reserved for refractory cases
![Page 36: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/36.jpg)
DRUGS & MGDrugs that may exacerbate MGAntibiotics :aminoglycosides,quinolones,macrolid
esNondepolarising muscle relaxants(curare)Beta blockersLocal anaesthetics &related agentsQuinine derivativesMagnesiumPenicillamine Botulinum toxins
![Page 37: A Case of Oro-Facio-Bulbar weakness](https://reader033.fdocuments.net/reader033/viewer/2022061116/54666adcaf795974338b5208/html5/thumbnails/37.jpg)
THANK YOU