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Medicine

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Neurology

Question
22 out of 25
 

Best Treatment of GBS is



A Dialysis
B Intravenous Steroids

C Plasmapheresis
D Vancomycin

Ans. C

Plasmapheresis

Guillain-Barre syndrome (Ref. Hari-18th ed., pg- 3473)

Definition and etiology. A predominantly demyelinating motor polyneuropathy. The illness can follow a nonspecific viral syndrome or be associated with HIV infection, Campylobacter jejuni infec­tion, hepatitis, infectious mononucleosis, Mycoplasma pneumoniae infection, vaccination, surgery, lymphoma, or SLE.

Diagnosis

1). Clinical signs

a. Classically, patients present with progressive ascending weakness and areflexia.

b. Generalized paralysis can develop gradually or relatively acutely, impeding respiratory function.

c. Relatively minor sensory signs and symptoms occur. Patients may complain of painful extremities.

d. The autonomic nervous system is often involved. Involvement of the autonomic nervous system can lead to early mortality as a result of cardiac arrhythmias and wide swings in blood pressure.

e. 'Bladder dysfunction may occur in severe cases but is usually transient.' (Bladder is most often spared then it is involved)

'If bladder dysfunction is a prominent feature and comes early in the course possibilities other than GBS should be considered particularly spinal cord disease ………. (Ref. Hari-18th ed., pg- 3476)

2). Diagnostic studies

a. Examination of the CSF shows an elevated protein and less than 50 mononuclear cells/mm3 (albuminocytologic dissociation).

b. The motor nerve conduction velocities are typically slowed.

c. Antiganglioside antibodies, most frequently to GM1, are common in GBS (20–50% of cases), particularly in those preceded by C. jejuni infection. (Ref. Hari- 18th ed., pg- 3474)

3). Therapy (Ref. Hari-18th ed., pg- 3477)

a. Plasmapheresis

b. Intravenous immunoglobulin.

Extra edge: (Ref. Hari-18th ed., pg- 3477)

Either high-dose intravenous immune globulin (IVIg) or plasmapheresis can be initiated, as they are equally effective for typical GBS. A combination of the two therapies is not significantly better than either alone.

In GBS 85% of the patients achieve a full functional recovery within several months to years.

Recovery is usually remarkable in GBS although all patients may not recover fully

Extra Edge: Plasmapheresis

1). Plasmapheresis is the removal, treatment, and return of (components of) blood plasma from blood circulation.

2). It is thus an extracorporeal therapy.

3). An important use of plasmapheresis is in the therapy of autoimmune disorders, where the rapid removal of disease-causing autoantibodies from the circulation is required

4). Other uses are the removal of blood proteins where these are overly abundant and cause hyperviscosity syndrome.

5). Diseases that can be treated with plasmapheresis:


a. Guillain-Barré syndrome

b. Chronic inflammatory demyelinating polyneuropathy (Onion bulb appearance on biopsy – AIIMS Nov 2010)

c. Goodpasture's syndrome

d. Hyperviscosity syndromes:

i. Cryoglobulinemia

ii. Paraproteinemia

iii. Waldenström macroglobulinemia

e. Myasthenia gravis

f. TTP

g. Wegener's granulomatosis

h. Lambert-Eaton Syndrome

i. Antiphospholipid Antibody Syndrome

j. Microscopic polyangiitis

k. Recurrent FSGS in the transplanted kidney

l. HELLP syndrome

m. Behcet syndrome

n. HIV-related neuropathy

o. Graves' disease in infants and neonates

p. Pemphigus vulgaris

q. Multiple sclerosis


Extra Edge: Plasmapheresis is not done in polymyositis.

Neurology Flashcard List

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