CASE PRESENTATION GBS By Dr. S. B. Sulehria Assistant Professor East Medical Ward KEMU/Mayo Hospital

BIODATA Name:Abdul waheed Age:25y Sex:male Religion:Muslim Address:Mughal poora.Lahore Mode:Emergency DOA:07/04/12

Presenting Complaints Pain in lower limbs=2weeks Weakness of both upper and lower limbs=1&1/2 week

HOPC My patient non-diabetic and non hypertensive was in his usual state of health 2 weeks back when he felt pain in lower limbs.pain was not localized,mild to moderate in intensity,no aggravating factor but relieved on taking medicaton.Then one and half week back patient started having weakness of both lower limbs which started from feet and and later involved legs and now weakness has progressed to both the upper limbs.The weakness is equal in both sides.

HOPC Cont…. Patient is unable to move his limbs even.There is no urinary or fecal incontinence.Patient has no h/o diarrhea or respiratory tract infection.There is no h/o fever.

Systemic Inquiry GIT: appetite normal,no change in bowel habits Resp: not significant CVS:no h/o orthopnea,dyspnea or chest pain Musculoskeletal system: no h/o joint pain or swelling. Muscular weakness is present

Past History No H/O hypertension,ischemic heart disease,diabetes,asthma,TB,Hepatitis

Family History Mother is hypertensive and diabetic No h/o ischemic heart disease,asthma or tuberculosis

Personal History Patient is not an addict,sleep and appetite normal,non smoker

Socioeconomic History Patient belongs to a poor family background

Differential Diagnosis Guillian Barre Syndrome Myelopathy Diphtheria Lyme disease Vasculitic neuropathy Botulism

General Physical Examination A young Male well oriented in time,place and person lying comfortably on the bed with vitals Pulse:72/min RR:16/min BP:100/60 Temp:99F Pallor -ve,jaundice-ve,facial puffiness and pedal edema-ve,clubbing,koilonychia- ve,lymph nodes not palpable

GIT Inspection: On inspection there abdomen is normal in shape,moving with respiration and there is no visible abnormality Palpation: On palpation there is no tenderness,rebound tenderness,and no palpable viscera Percussion:No shifting dullness or fluid thrill present Auscultation:Normal bowel sounds

CVS S1+S2+0 No added heart sound or murmur heard

Respiratory System Normal Vesicular breathing with no added sound

CNS GCS: 15/15 Higher mental functions are intact Speech is normal

Motor System Bulk is b/l normal and comparable in all four limbs Tone is decreased in all four limbs Power is 0/5 in both the lower limbs and 1/5 in both the upper limbs Deep tendon reflexes are absent Plantars are b/l non responsive

Sensory System Intact Cerebellum: Normal SOMI: negative Cranial Nerves: intact Pupils are b/l reactive

INVESTIGATIONS CBC: HB:12.4g/dl WBC:9700 PLT:458000/mm3 HCT:38.7 MCV:86.7 MCH:34 MCHC:34.5

INVESTIGATIONS (Cont…) LFTs: ALT:27 AST:38 ALP:276 T.BIL:1.0 RFTs:Normal U/C: Normal

INVESTIGATIONS (Cont….) USG Abdomen: Normal CXR: Normal CSF Analysis: Proteins: 250mg/dl Glucose:67mg/dl Cells:5/mm3 all lymphocytes EMG and NCS: acute polyneuropathy with predominantly demyelinating features

Final Diagnosis

Guillian Barre Syndrome

Introduction It is an acute, frequently severe and fulminant polyradiculoneuropathy that is autoimmune in nature. Males are at 1.5 times high risk for GB syndrome than females Incidence is 1 case per million per month in USA and Canada

Antecedent Facts App 70% of cases have a h/o respiratory tract or the gastrointestinal infections Compylobacter Jejuni is cultured in upto 30% of the cases Other possible associations are with CMV, EBV, HHV, Mycoplasma pneumoniae Also associated with lymphomas, HIV seropositive individuals and SLE.

Symptoms and Signs Complaint of weakness often having a proximal emphasis and symmetric distribution. Frequently involving arms and often one or both sides of the face. Muscles of respiration and deglutition may also be affected. Sensory symptoms include distal parasthesias and dysesthesias.

Autonomic Disturbances Common and may be severe Tachycardia Cardiac irregularities Hypo or hypertension Abnormalities of sweating,pulmonary dysfunction and impaired sphincter control

Subtypes of GBS SubtypeFeaturesElectrodiagn osis AIDP( Acute inflammatory demyelinating polyneuropathy) Adults affected more than children,rapid recovery, anti GM1 antibodies(<50%) Demyelinating AMAN( Acute motor axonal neuropathy) Children and young adults may be seasonal recovery rapid, anti GD1a antibodies Axonal AMSAN(Acute motor sensory axonal neuropathy) Mostly adults uncommon and recovery is slow and often incomplete closely related to AMAN Axonal Miller fisher syndromeAdults and children, uncommon, ophthalmoplegia,ataxi a, and areflexia anti GQ1b antibodies Demyelinating

Laboratory Features CSF findings Raised proteins( mg/dl) Transient increase in CSF WBCs

EMG and NCS Demyelinating types: prolonged distal latencies, conduction velocity slowing, evidence of conduction blocks and temporal dispersion of compound action potentials. Axonal types: Reduced amplitude of the compound action potentials

Diagnostic Criteria For GBS Required: 1.progressive weakness of 2 or more limbs due to neuropathy 2.Areflexia 3.Disease course < 4 weeks 4.Exclusion of other causes(e.g vasculitis,toxins,botulism,diphtheria, porphyria,localized spinal cord or cauda equina syndrome

Diagnostic Criteria For GBS Supportive 1. Relatively symmetrical weakness 2. Mild sensory involvement 3. Facial or other cranial N involvement 4. Absence of fever 5. Typical CSF profile 6.Electrophysiologic evidence of demyelination

Treatment IVIG 400mg per kg per day for five days Plasmapharesis 40-50ml per kg plasma exchange four times over a week. Glucorticoids have not been found effective.

Ventilatory Support Decreasing forced vital capacity Intubation considered if FVC< 15ml/kg Dyspnea evident Oxygen saturation is decreasing Low dose heparin to prevent PE

THANKS