GUILLAIN-BARRE SYNDROME

GBS
Eponym that encompasses acute immunemediated polyneuropathies Peripheral nerve myelin is target of an immune attack Starts at level of nerve root=conduction blocks & muscle weakness Eventually get widespread patchy demyel= increased paralysis

PATHOPHYSIOLOGY
Usually

postinfectious Immune-mediated: infectious agents thought to induce Ab production against specific gangliosides/glycolipids Lymphocytic infiltration of spinal roots/peripheral nerves & then macrophagemediated, multifocal stripping of myelin Result: defects in the propagation of electrical nerve impulses, with eventual conduction block and flaccid paralysis

CLINICAL FEATURES:

Progressive, fairly symmetric muscle weakness -typically starts in proximal legs -10% will 1st develop weakness in face or arms -severe resp muscle weakness in 10-30% pts -oropharyngeal weakness in ~ 50%

Absent or depressed DTR Often prominent severe pain in lower back Common to have paresthesias in hands and feet Dysautonomia is very common: tachycardia, urinary retention, hypertenison alternating w/ hypotension, ileus

DIAGNOSIS:
Albuminocytologic dissociation: elevated CSF protein w/ normal WBC (80-90% pts) Electromyography (EMG) helps confirm diagnosis = prolonged or absent F waves

NINDS EXPERT CONSENSUS:

1.
2.

Reqd Features for dx: Progressive weakness of > than 1 limb Areflexia Supportive Features: ~progression of Sx over days to 4 weeks ~relative symmetry ~CN involv esp b/l facial n weakness ~autonomic dysfunction ~EMG features ~elev CSF protein w/ cell count ,10 mm3

GBS=HETEROGENOUS SYNDROME W/
VARIANT FORMS
Think

of AIDP as the traditional form as described previously, accts for 85-90% Miller Fisher Syndrome: opthalmoplegia, ataxia, and areflexia (5%). GQ1b antibody. Only 1/4th w/ extremity weakness AMAN: selective involv of motor nerves, DTRs are preserved, more common in Japan/China, almost all preceded by Campylobacter infxn AMSAN: more severe form of AMAN +sensory

DDX OF POLYNEUROPATHY:
Arsenic

poisoning N-Hexane (glue sniffing) Vasculitis Lyme Disease Tick paralysis Sarcoidosis Leptomeningeal Dz Paraneoplastic Dz Critical Illness

SUPPORTIVE CARE
Monitor Resp status closely (follow NIFs), up to 30% may req ventilatory support In severe cases, intrarterial monitoring may be necessary given the gisngifcant blood pressure fluctuations Neuropathic pain plagues most, often managed w/ Gabapentin or Carbamazepine

DISEASE MODIFYING TREATMENT

IVIG

: typically given for 5 d at 0.4 gram/kg/d (may need to extend course depending on response) Plasmapheresis: usually 4-6 treatments over 8-10 days The choice b/w plasma exchange and IVIG is dep on availability, pt contraindications, etc. Because of ease of administration, IVIG is frequently preferred. The cost and efficacy of the 2 treatments are comparable. Glucocorticoids have NO ROLE!!

OUTCOMES:
65% can walk independently @ 6 mos Overall, 80% usually recover completely 5-10% have prolonged course w/ incomplete recovery, ~3% wheelchair bound Approx 5% die despite ICU care 2% will develop chronic relapsing Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

REFERENCES:
Plasmapheresis

and acute Guillain-Barre syndrome. The Guillain-Barre Syndrome Study Group. Neurology 1984; 2:1296.

Ropper, AH. The Guillain-Barre Syndrome. N Engl J Med 1992; 326:1130. Sumner, AJ. The physiologic basis for symptoms in Guillain-Barre Syndrome. Ann Neurol 1981; 9 Suppl:28.

TICKS PARALYSIS
Tick paralysis is the only tick-borne disease that is not caused by an infectious organism. The illness is caused by a neurotoxin produced in the tick's salivary gland. After prolonged attachment, the engorged tick transmits the toxin to its host. The incidence of tick paralysis is unknown. Clinical features: Beginning with weakness in both legs that progresses to paralysis. The paralysis ascends to the trunk, arms, and head within hours and may lead to respiratory failure and death. The disease can present as acute ataxia without muscle weakness. Patients may report minor sensory symptoms, but constitutional signs are usually absent. Deep tendon reflexes are usually hypoactive or absent, and ophthalmoplegia and bulbar palsy can occur.

TICK

CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (CIDP)

Both GBS and CIDP are similar most, there are shuttle changes. Similarities an increased CSF protein a demyelinative type of nerve conduction abnormality probable autoimmune pathogenesis, and an inflammatory pathology.

DIFFERENCES

A preceding illness is relatively uncommon in patients with CIDP. Whereas GBS is an acute (rarely sub acute) monophasic illness CIDP evolves more slowly either in a steadily progressive or stepwise manner (sometimes in an asymmetric pattern) and attains its maximum severity only after weeks, months or longer, following which it tends to run a relapsing or fluctuating course. Most cases of CIDP respond favourably to the prolonged administration of corticosteroids, as well as to plasma exchange and, in many cases, to intravenous to immune globulin

PERONEAL MUSCULAR ATROPHY (CHARCOTMARIE-TOOTH DISEASE)

Inherited neuropathy Autosomal dominant trait Onset Late childhood or adolescence, with atrophy of muscles of the feet and legs and later of the hands and arms

CLINICAL FEATURES
The early involvement of the peronei and extensors of the toes produces an equinovarus deformity and clawfoot. Deep and superficial sensation are impaired, usually to a slight degree, and tendon reflexes are absent in the affected limbs. The illness progresses very slowly, with long periods of stability. Wasting seldom extends above the elbows and the lower third of the thighs. Sensory ataxia and weakness --- instability in gait

DIABETIC NEUROPATHIES
Diabetic ophthalmoplegia Acute mononeuropathy Lumbar mononeuropathy multiplex proximal diabetic neuropathy distal sensory type autonomic involvement Segmental radiculopathy

DIABETIC OPHTHALMOPLEGIA
Infarction of the 3rd or 6th cranial nerve Occular palsy associated with severe pain around the eye and forehead Pupillary constriction ??????

The pupilloconstrictor fibers, located peripherally in the third nerve, are spared by the infarction, which characteristically affects the central portion of the nerve; hence, pupillary function is usually intact

ACUTE MONONEUROPATHY

Femoral and sciatic nerve most commonly affected

LUMBAR MONONEUROPATHY MULTIPLEX

Diabetic amyotrophy subacutely evolving, painful, asymmetric or unilateral, predominantly motor neuropathy, affecting multiple lumbosacral nerves. Muscle weakness and atrophy are most evident in thepelvic girdle and thigh muscles on one side and the knee jerk is lost. Recovery time more months

PROXIMAL DIABETIC NEUROPATHY I symmetric weakness and wasting of the pelvic and proximal thigh muscles, of insidious onset and gradual evolution. Scapular and upper arm muscles are affected less frequently. Pain is not a consistent feature, and sensory changes, if present, are mild and of the distal symmetric type.

DISTAL SENSORY TYPE This takes the form of persistent and often distressing pain, numbness, and tingling, affecting the feet and lower legs symmetrically. In severe cases, the hands may be affected. Occasionally deep sensation is impaired, with ataxia and bladder atony (diabetic pseudotabes)

AUTONOMIC INVOLVEMENT pupillary and lacrimal dysfunction, impairment of sweating and vascular reflexes, nocturnal diarrheoa, atonicity of the gastrointestinal tract and bladder, impotence, and postural hypotension. These symptoms are frequently combined with other forms of diabetic neuropathy, particularly with the distal sensory type

SEGMENTAL RADICULOPATHY
common complication of long-standing diabetes, presenting with severe pain, dysesthesia, and superficial sensory loss in a segmental distribution over the chest or abdomen. The EMG changes (fibrillations of paraspinal muscles in multiple myotomes) confirm the presence of a widespread radiculopathy