Guillain–Barré syndrome

The paralysis of Guillain- Barre occurs about 10 days after a nonspecific viral illness. Weakness is gradual over days or weeks, beginning in the lower extremities and progressing toward the trunk. Later, the upper limbs and the bulbar muscles can become involved. Involvement of the respiratory muscles is life threatening. The syndrome seems to be caused by a demyelination in the motor and, occasionally, the sensory erves. Measurement of spinal fluid protein is helpful in the diagnosis; protein levels are increased to more than twice normal, while glucose and cell counts are normal. Hospitalization for observation is indicated. Treatment can consist of intravenous immunoglobulin, steroids, or plasmapheresis. Recovery is not always complete. Bell palsy usually follows a mild upper respiratory infection, resulting in the rapid development of weakness of the entire side of the face. Muscular dystrophy encompasses a number of entities that include weakness over months. Charcot- Marie-Tooth disease has a clinical onset including perineal and intrinsic foot muscle atrophy, later extending to the intrinsic hand muscles and proximal legs.

• In the vast majority of patients with GBS, treatment should be initiated as soon after diagnosis as possible.
• 2 weeks after the first motor symptoms, immunotherapy is no longer effective.
• Either high-dose intravenous immune globulin (IVIg) or plasmapheresis can be initiated, as they are equally effective.
• A combination of the two therapies is not significantly better than either alone.
• Glucocorticoid administration does not shorten the course or prognosis of GBS.

Answer is C (Sensory level):

Guillion Barre syndrome is not associated with a distinct sensory level.

Sensory involvement in GBS is mild. Although sensory symptoms (tingling/paraesthesias) may be prominent, cutaneous sensory deficits (eg loss of pain and temperature sensations) are usually mild (Harrison’s) or even absent (Current Neurology).

Guillain Barre syndrome (GBS) is an autoimmune disease characterized by acute demyelinating polyneuropathy.

It has several distinctive features, which are so often asked. These include :

Aetiology

Preceding evidence of a viral infection such as that with –   Herpes group : CMV / EBV, Campylobacter jejuni gastroenteritis, Lymphoma / Lupus Erythematosus

Clinical manifestation

1. Predominant motor involvement

• Paraplegia / Quadriplegia
• Proximal muscles are affected more than distal ones.
• Ascending pattern of paralysis
• Cranial nerve involvement 9:  The commonest involvement is bilateral Facial and then Bulbar muscles. The patient has difficulty in swallowing, speech, etc.
• Areflexia -Deep tendon reflexes usually disappear within the first few days of onset.

2.    Sensory disturbance

• Sensory disturbance may or may not be present : Cutaneous sensory deficits (eg.loss of pain and temperature sensation)when present are usually mild.
• The bladder & Bowel rarely involved.
• Bladder dysfunction may occur in severe cases but is usually transient
• If bladder dysfunction is a prominent feature and comes early, possibilities other than GBS should be considered

3.    Autonomic

• Characterized by Autonomic involvement Postural hypotension. episodes of hypotension or hypertension and tachycardia.

CSF Findings

– CSF shows albumino-cytological dissociation i.e. Increased proteins with normal cells

– CSF Pleocytosis with cells above 50/mm³ is against the diagnosis^.GBS. 

Nerve Conduction Studies

Nerve conduction studies show demyelination i.e. slow conduction velocity, distal latency, conduction block

Prognosis

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

– Approximately 85% of patients with GBS achieve a full functional recovery within several months to a year.

Answer is D (Bladder bowel involvement):

Ans. C: Descending paralysis

There is an elevation of the CSF protein due to inflammation and altered permeability of the microcirculation within the spinal roots as they traverse the subarachnoid space.

Inflammatory cells are contained within the roots, however, and there is little to no CSF pleocytosis.

Ans. B i.e. Guillain Barre syndrome

Guillain Barre syndrome

Ascending type of motor paralysis

• Arreflexia
• Flaccidity
• Albumino-cytological dissociation in CSF

Treatment:

– Plasmapheresis

– Immunoglobulin

Ans. B: Loss of limb reflex

In Guillain-Barré syndrome, the body’s immune system attacks part of the peripheral nervous system.

The syndrome can affect the nerves that control muscle movement as well as those that transmit pain, temperature and touch sensations.

This can result in muscle weakness and loss of sensation in the legs and/or arms.

Ans. D: Facial
Signs and symptoms may take days or weeks to develop and days, months, or even years to fully disappear. The severity of a patient’s condition depends on the extent of nervous system involvement-that is the extent of demyelination. Cranial nerve involvement is present in 85% of cases, with the facial nerve (cranial nerve VII) most often affected. The glossopharyngeal (cranial nerve IX), vagus (cranial nerve X), spinal accessory (cranial nerve XI), and hypoglossal (cranial nerve XII) nerves also may be affected.
Signs and symptoms of facial nerve dysfunction include the inability to smile, frown, whistle, or use a straw when drinking. Paralysis of cranial nerves IX and X can cause dysphagia and laryngeal paralysis. Autonomic dysfunction may occur if the vagus nerve is affected.
Motor weakness and paralysis usually begin in the legs, progressing to the trunk and arms. If sensory symptoms are present, patients may complain of paresthesias and pain.
After the first few days of weakness and paresthesias, a physical assessment will reveal diminished (hyporeflexia) or absent deep tendon reflexes (areflexia) in the involved extremities.
Areflexia is considered a key assessment finding in GBS.
Respiratory failure is thought to be caused by involvement of the muscles of respiration, including the diaphragm and the intercostals.
GBS doesn’t affect the patient’s cognitive function, level of consciousness, or pupillary signs.
Autonomic dysfunction-signs and symptoms include cardiac dysrhythmias, paroxysmal hypotension, orthostatic hypotension, paralytic ileus, urinary retention, and syndrome of inappropriate secretion of antidiuretic hormone.

Answer- A, B, C, D, Areflexia, Progressive weakness of at least 2 limbs, Exclusion of vasculitis, Albumin-cytological dissociation

Diagnostic criteria for Guillain Barre syndrome-

Required-

• Progressive weakness of 2 or more limbs due to neuropathy.
• Areflexia.
• Exclusion of other causes [e.g. vasculitis].

Supportive-

• Mild sensory involvement.
• Facial nerve or other cranial nerve involvement.
• Typical C.S.F profile (albumino- cytological dissociation).
• Absence of fever.

Ans.A. Steroids

• Guillain-Barré syndrome is one of the most common life-threatening diseases of the peripheral nervous system.
• It is a rapidly progressive acute demyelinating disorder affecting motor axons that result in ascending weakness that may lead to death from failure of respiratory muscles over a period of only several days. It appears to be triggered by an infection or a vaccine that breaks down self-tolerance, thereby leading to an autoimmune response.

Treatments include :

• plasmapheresis (to remove offending antibodies),
• intravenous immunoglobulin infusions (which suppress immune responses through unclear mechanisms) and
• supportive care, such as ventilatory support. Patients who survive the initial acute phase of the disease usually recover over time.

• In patients with Guillain Barre syndrome, there is an elevation of the CSF protein due to inflammation and altered permeability of the microcirculation within the spinal roots as they traverse the subarachnoid space.
• Inflammatory cells are contained within the roots, however, and there is little to no CSF pleocytosis.
• This is termed as albumin-cytological dissociation.

Ans. D

Causes of acute quadriparesis:

· Brainstem ischemia

· Cervical cord ischemia

· Trauma

· Electrolyte abnormality

· GBS

· Myasthenia

· Snakebite, botulism, diphtheria

Causes of subacute and chronic quadriparesis:

· Motor neuron disease

· Myopathy

Answer- B

GBS, a rare but serious autoimmune illness, affects the nervous system and causes acute flaccid paralysis. GBS can occur as a sequel to Campylobacter spp. infection 10 days–3 weeks after gastrointestinal illness.

Infection with Campylobacter jejuni, which causes diarrhea, is one of the most common causes of GBS.

Pathogenesis:-Campylobacter is thought to cause this autoimmune disease through a mechanism called molecular mimicry, whereby Campylobacter contains ganglioside-like epitopes in the lipopolysaccharide moiety that elicit autoantibodies reacting with peripheral nerve targets.

Treatment of Campylobacter JeJuni is supportive care. For severe cases, antibiotics, such as azithromycin and quinolones, may be indicated early in the diarrheal disease where Guillain-Barré syndrome can be a sequel