Guillain-Barre Syndrome

 is a postinfectious polyneuropathy involving mainly motor  sometimes also sensory and autonomic nerves.  This syndrome affects people of all ages and is not hereditary.  Most patients have a demyelinating neuropathy, but primarily axonal degeneration is documented in some cases

Etiology :-  The paralysis usually follows a nonspecific viral infection by about 10 days.  The original infection might have caused only gastrointestinal (especially Campylobacter jejuni, but also Helicobacter pylori) or  respiratory tract (especially Mycoplasma pneumoniae) symptoms.  West Nile virus also can cause Guillain-Barre-like syndrome

 Guillain-Barre syndrome is reported following administration of vaccines against rabies, influenza, and poliomyelitis (oral) and  following administration of conjugated meningococcal vaccine, particularly serogroup C. Etiology :-

Clinical Manifestations :- Weakness usually begins in the lower extremities and progressively involves the trunk, the upper limbs, and finally the bulbar muscles, a pattern known as Landry ascending paralysis. Proximal and distal muscles are involved relatively symmetrically, asymmetry is found in 9% of patients. Tendon reflexes are lost, usually early in the course, but are sometimes preserved until later. in cases with an abrupt onset, tenderness on palpation and pain in muscles is common in the initial stages. Weakness can progress to inability or refusal to walk and later to flaccid tetraplegia. Paresthesias occur in some cases. Some young patients exhibit symptoms of viral meningitis or meningoencephalitis.

Bulbar involvement occurs in about half of cases. Dysphagia and facial weakness are often impending signs of respiratory failure. They interfere with eating and increase the risk of aspiration.

Autonomic nervous systemInvolvement :- Autonomic nervous system Involvement :- in some cases. Lability of blood pressure and, postural hypotension, Lability of HR, and episodes of profound bradycardia, and occasional asystole occur. **SO Cardiovascular monitoring is important.. Urinary incontinence or retention of urine is a complication in about 20% of cases but is usually transient.

Variants of GBS :- Variants of GBS :- Landry ascending paralysis I.Classic type Landry ascending paralysis, is the most common II.Miller-Fisher syndrome consists of acute external ophthalmoplegia, ataxia, and areflexia. III.Chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) :that recur intermittently or do not improve or progress slowly for periods of months to years. IV.Congenital GBS is described rarely, improve gradually and donot require tx.

DIFFERENTIAL DIAGNOSIS OF CHILDHOOD GBS : SPINAL CORD LESIONS Acute transverse myelitis Epidural abscess Tumors Poliomyelitis Vascular malformations Cord infarction Fibrocartilaginous embolism Cord compression from vertebral subluxation related to congenital abnormalities or trauma Acute disseminated encephalomyelitis Hopkins syndrome

DIFFERENTIAL DIAGNOSIS OF CHILDHOOD GBS : PERIPHERAL NEUROPATHIES  Toxic Vincristine Glue sniffing Heavy metal Organophosphate pesticides  Infections HIV Diphtheria Lyme disease  Inborn errors of metabolism Tangier diseas Porphyria

 NEUROMUSCULAR JUNCTION DISORDERS Tick paralysis, myasthenia gravis, botulism, hypercalcemia Periodic paralyses, dermatomyositis Myopathies DIFFERENTIAL DIAGNOSIS OF CHILDHOOD GBS :

Laboratory Findings and Diagnosis :- Laboratory Findings and Diagnosis :- The CSF protein is elevated to more than twice the upper limit of normal, glucose level is normal, and there is no pleocytosis. Fewer than 10 white blood cells/mm 3 are found. The results of bacterial cultures are negative, and viral cultures rarely isolate specific viruses. ** ** The dissociation between high CSF protein and a lack of cellular response in a patient with an acute or subacute polyneuropathy is diagnostic of Guillain-Barre syndrome.

MRI of the spinal cord may be indicated to rule out other disorders.MRI findings include thickening of the cauda equina and intrathecal nerve roots with gadolinium enhancement. These finds are fairly sensitive and are present in >90% of patients Motor NCVs are greatly reduced sensory nerve conduction time is often slow. Electromyography (EMG) shows evidence of acute denervation of muscle. Serum creatine kinase (CK) level may be mildly elevated or normal Antiganglioside antibodies, mainly against G M1 and G D1, are sometimes elevated in the serum in Guillain-Barre syndrome

Muscle biopsy is not usually required for diagnosis; specimens appear normal in early stages and show evidence of denervation atrophy in chronic stages. Serologic testing for Campylobacter and Helicobacter infections helps establish the cause if results are positive but does not alter the course of treatment. Results of stool cultures are rarely positive because the infection is self-limited and only occurs for about 3 days, and the neuropathy follows the acute gastroenteritis.

Treatment :- Treatment :-  Patients in early stages of this acute disease should be admitted to the hospital for observation because the ascending paralysis can rapidly involve respiratory muscles during the next 24 hr.  Patients with slow progression might simply be observed for stabilization and spontaneous remission without treatment. A commonly recommended protocol is IVIG 0.4 g/kg/day for 5 consecutive days.  Rapidly progressive ascending paralysis is treated with intravenous immunoglobulin (IVIG), administered for 2, 3, or 5 days. A commonly recommended protocol is IVIG 0.4 g/kg/day for 5 consecutive days.

Treatment :- Treatment :-  Plasmapheresis and/or immunosuppressive drugs are alternatives if IVIG is ineffective. Steroids are not effective.  Combined administration of immunoglobulin and interferon is effective in some patients.  Supportive care, such as respiratory support, prevention of decubiti in children with flaccid tetraplegia  treatment of secondary bacterial infections, is important gabapentin  For the treatment of chronic neuropathic pain following GBS gabapentin is more effective than carbamazepine, and the requirement for fentanyl is reduced.

Tx. of CIDPs :- Tx. of CIDPs :- whether relapsing-remitting or unremitting,  treated with oral or pulsed steroids or with IVIG. S.C IG infusion may be an alternative to the IV route.  Plasma exchange, sometimes requiring as many as 10 exchanges daily, is an alternative  relapses usually respond to another course of plasmapheresis. Steroid and immunosuppressive drugs are another alternative, but their effectiveness is less predictable. High-dose pulsed methylprednisolone given intravenously is successful in some cases. N.B ; Even if C. jejuni infection is documented by stool culture or serologic tests, treatment of the infection is not necessary because it is self-limited, and the use of antibiotics does not alter the course of the polyneuropathy.

Prognosis Prognosis  The clinical course is usually benign, and spontaneous recovery begins within 2-3 wk.  Most patients regain full muscular strength, although some are left with residual weakness.  The tendon reflexes are usually the last function to recover.  Improvement usually follows a gradient opposite the direction of involvement: bulbar function recovering first, and lower extremity weakness resolving last.  The electrophysiologic features of conduction block are predictive of good outcome

cranial nerve involvement, intubation, and maximum disability at the time of presentation.  3 clinical features are predictive of poor outcome with sequelae: cranial nerve involvement, intubation, and maximum disability at the time of presentation.  The prognosis in chronic forms of the Guillain- Barre syndrome is more guarded than in the acute form, and many patients are left with major residual handicaps. Prognosis Prognosis

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