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Syndromes Presenting as Acute Flaccid Paralysis

Flaccid paralysis evolving over hours or a few days suggests involvement of the lower motor neuron complex (see section on Floppy Infant Syndrome). Anterior horn
cells (spinal cord) may be involved by viral infection (paralytic poliomyelitis) or by paraviral or postviral immunologically mediated disease (acute transverse myelitis).
The nerve trunks (polyneuritis) may be diseased as in Guillain-Barré syndrome or affected by toxins (diphtheria or porphyria). The neuromuscular junction may be
blocked by tick toxin or botulinum toxin. The paralysis rarely will be due to metabolic (periodic paralysis) or inflammatory muscle disease (myositis). A lesion
compressing the spinal cord must be ruled out.

Clinical Findings

Symptoms and Signs

Features assisting diagnosis are age, a history of preceding or waning illness, the presence (at time of paralysis) of fever, rapidity of progression, cranial nerve findings,
and sensory findings (Table 23–25). The examination may show long tract findings (pyramidal tract), causing increased reflexes and a positive Babinski sign. The
spinothalamic tract may be interrupted, causing loss of pain and temperature. Back pain, even tenderness to percussion, may occur, as well as bowel and bladder
incontinence. Often the paralysis is ascending, symmetric, and painful (muscle tenderness or myalgia). Laboratory findings occasionally are diagnostic.

Table 23–25. Acute Flaccid Paralysis in Children.


Poliomyelitis (Paralytic, Landry-Guillain-Barré Botulism Tick-Bite Paralysis Transverse Myelitis and
Spinal, and Bulbar), With Syndrome ("Acute Idiopathic Neuromyelitis Optica
or Without Encephalitis Polyneuritis")
Etiology Poliovirus types I, II, and Likely delayed hypersensitivity— Clostridium botulinum toxin. Probable interference Usually unknown; multiple
III; other enteroviruses, eg, with T-cell–mediated Block at neuromuscular with transmission of viruses (herpes, EBV,
EV71; vaccine strain polio antiganglioside antibodies. junction. Under age 1, toxin nerve impulse caused by varicella, hepatitis A) often
virus (rare); West Nile virus: Mycoplasmal and viral infections synthesized in bowel by toxin in tick saliva. postviral (see Guillain–
epidemic in birds. (EBV, CMV), Campylobacter organisms in ingested dust or Barré syndrome).
Mosquitoes infect horses, jejuni, Hepatitis B. honey. At older ages toxin
humans ingested in food. Rarely from
wound infection.
History None, or inadequate polio Nonspecific respiratory or Infancy: dusty environment Exposure to ticks (dog Rarely symptoms
immunization. Upper gastrointestinal symptoms in (eg, construction area), tick in eastern United compatible with multiple
respiratory or preceding 5–14 d common. Any honey. Older: food States; wood ticks). sclerosis or optic neuritis.
gastrointestinal symptoms season, though slightly lower poisoning. Multiple cases Irritability 12–24 h before Progression from onset to
followed by brief respite. incidence in summer. hours to days after ingesting onset of a rapidly paraplegia often rapid,
Bulbar paralysis more contaminated food. progressive ascending usually without a history of
frequent after tonsillectomy. paralysis. bacterial infection.
Often in epidemics, in
summer and early fall.
Presenting Febrile at time of paralysis. Symmetrical weakness of lower Infancy: constipation, poor Rapid onset and Root and back pain in about
complaints Meningeal signs, muscle extremities, which may ascend suck and cry. "Floppy." progression of ascending one-third to one-half of
tenderness, and spasm. rapidly to arms, trunk, and face. Apnea. Lethargy. Choking flaccid paralysis; often cases. Sensory loss below
Asymmetrical weakness Verbal child may complain of (cause of SIDS?). Older: accompanied by pain and level of lesion
widespread or segmental paresthesias. Fever uncommon. blurred vision, diplopia, paresthesias. Paralysis of accompanying rapidly
(cervical, thoracic, lumbar). Facial weakness early. Miller- ptosis, choking, weakness. upper extremities second developing paralysis.
Bulbar symptoms early or Fisher variant presents as ataxia day after onset. Sphincter difficulties
before extremity weakness; and ophthalmoplegia (rare). Sometimes acute ataxia common. Fever (58%).
anxiety; delirium. presentation.
Findings Flaccid weakness, usually Flaccid weakness, symmetric, Infants: Flaccid weakness. Flaccid, symmetric Paraplegia with areflexia
asymmetrical. Cord level: usually greater proximally, but Alert. Eye, pupil, facial paralysis. Cranial nerve below level of lesion early;
Lumbar: legs, lower may be more distal or equal in weakness. Deep tendon and bulbar (respiratory) later, may have
abdomen. Cervical: distribution. Rarely cranial nerves reflexes decreased. Absent paralysis, ataxia, hyperreflexia. Sensory loss
shoulder, arm, neck, IX–XI, III–VI. Miller-Fisher suck, gag. Constipation. sphincter disturbances, below and hyperesthesia or
diaphragm. Bulbar: variant: ophthalmoplegia, ataxia. Older: paralysis and sensory deficits may normal sensation above
CMV, cytomegalovirus; CSF, cerebrospinal fluid; EBV, Ebstein–Barr virus; IVIg, intravenous immune globulin; MRI, magnetic resonance imaging; PMN,
polymorphonuclear neutrophil; SIDS, sudden infant death syndrome.

Laboratory Findings

(See Table 23–25.) Examination of CSF is helpful. Imaging studies of the spinal column (plain radiographs) and spinal cord (MRI) are occasionally essential. Viral
cultures (CSF, throat, and stool) and titers aid in diagnosing poliomyelitis. A high sedimentation rate may suggest tumor or abscess; the presence of antinuclear antibody
may suggest lupus arteritis.

EMG and nerve conduction velocity can be helpful in diagnosing polyneuropathy. Nerve conduction is usually slowed after 7–10 days. Findings in botulism and tick-bite
paralysis can be specific and diagnostic. Rarely, elevation of muscle enzymes or even myoglobinuria may aid in diagnosis of myopathic paralysis. Porphyrin urine studies
and heavy-metal assays (arsenic, thallium, and lead) can reveal those rare toxic causes of polyneuropathic paralysis.

Differential Diagnosis

The child who has been well and becomes paralyzed often has polyneuritis. Acute transverse myelitis sometimes occurs in an afebrile child. The child who is ill and
febrile at the time of paralysis often has acute transverse myelitis or poliomyelitis. Acute epidural spinal cord abscess (or other compressive lesion) must be ruled out.
Poliomyelitis is very rare in our immunized population. Enterovirus 71 and West Nile disease are two new causes. Paralysis due to tick bites occurs seasonally (spring and
summer). The tick is usually found in the occipital hair. Removal is curative.

Paralysis due to botulinum toxin occurs most commonly in those younger than age 1 year (see Chapter 38). Intravenous drug abuse can lead to myelitis and paralysis.
Furthermore, chronic myelopathy occurs with two human immunodeficiency virus infections: HTLV-I and HTLV-III (now called HIV-1).

Complications

Respiratory Paralysis

Early and careful attention to oxygenation is essential. Administration of oxygen, intubation, mechanical respiratory assistance, and careful suctioning of secretions may
be required. Increasing anxiety and a rise in diastolic and systolic blood pressures are early signs of hypoxia. Cyanosis is a late sign. Deteriorating spirometric findings
(forced expiratory volume in 1 second and total vital capacity) may indicate the need for controlled intubation and respiratory support. Blood gases (usually late changes
with increased CO2 and decreased O2) can aid decisions.

Infections
Pneumonia is common, especially in patients with respiratory paralysis. Antibiotic therapy is best guided by results of cultures. Bladder infections occur when an
indwelling catheter is required because of bladder paralysis. Recovery from myelitis may be delayed by urinary tract infection.

Autonomic Crisis

This may be a cause of death in Guillain-Barré syndrome. Strict attention to vital signs to detect and treat hypotension or hypertension and cardiac arrhythmias in an
intensive care setting is advisable, at least early in the course and in severely ill patients.

Treatment

Most of these syndromes have no specific treatment. Ticks causing paralysis must be removed. Other therapies include the use of erythromycin in Mycoplasma infections
and botulism immune globulin in infant botulism. Recognized associated disorders (eg, endocrine, neoplastic, or toxic) should be treated by appropriate means. Supportive
care also involves pulmonary toilet, adequate fluids and nutrition, bladder and bowel care, prevention of decubitus ulcers, and in many cases, psychiatric support.

Corticosteroids

These agents are believed by most to be of no benefit in Guillain-Barré syndrome. Autonomic symptoms (eg, hypertension) in polyneuritis may require treatment.

Plasmapheresis, Intravenous Immunoglobulin

Plasma exchange or intravenous IgG has been beneficial in moderate or severe cases of Guillain-Barré syndrome. Some clinicians use inability to ambulate as a criterion
to use IVIg.

Physical Therapy

Rehabilitative measures are best instituted when acute symptoms have subsided and the patient is stable.

Antibiotics

Appropriate antibiotics and drainage are required for epidural abscess.