Acute flaccid paralysis Dr Nebal Waill CWTH / Pediatric neurology department

Definition Acute Flaccid Paralysis (AFP) occurs when there is rapid evolution of motor weakness (< than 4 days), with a loss of tone in the paralysed limb. This excludes weakness due to trauma and spastic paralysis. AFP is a medical emergency as unnecessary delays can result in death and disability

The list of underlying causes of AFP is broad, and there is substantial variation by age, ethnicity, and geographic area. In the absence of wild virus-induced poliomyelitis, the acute demyelinating form of Guillain-Barre syndrome (AIDP) accounts for at least 50 percent of AFP cases globally followed in frequency by paralytic non-polio enterovirus infection, the motor axonal form of Guillain-Barre syndrome (AMAN), traumatic neuritis, and acute transverse myelitis.

Background • 1916- Guillian, Barre and Strohl described 2 French soldiers with motor weakness, areflexia, and “albuniocytological dissociation” in the cerebrospinal fluid. They recognized the peripheral nature of the illness. Guillian Barre Andre Strohl

Epidemiology Annual incidence of GBS = 1-3/ 100000 persons annually. Rare in infants. Male & female have similar risk Any age but most frequent at 4-9 years

GBS subtypes Sporadic AIDP AMSAN AMAN MFS

Pathology Both motor and sensory fibers are affected AIDP Segmental demyelination occurs at all levels of peripheral nervous system CNS alterations are secondary to axonal degeneration and affect Segmental demylination Axonal degeneration ( less extensive ) Anterior + posterior roots Sympathatic chain and ganglia peripheral nerves Anterior horn cells in spinal grey matter Neurons of motor cranial nerves neucli in brainstem

Pathogenesis

Pathogenesis myelin sheath node of Ranvier myelin sheath

Documented in 2/3 of cases Antecedent Events Documented in 2/3 of cases Agents : Campylobacter jujeni CMV EBV HSV H.Influenzae Mycoplasma Vaccines : Rabies Swin-flu Tetanus Mumps Measles Rubella Hepatitis A Hepatitis B H. Influenzae type b Trauma Sugrical procedures C.Jujeni accounts for 1/3 of GBS because of mimicry between gangliosides and lipopolysaccharides of the bacteria

Clinical features AIDP Infection (GIT: Campylobacter, Respiratory: Mycoplasma) within 2 weeks of onset Weakness(lower extremities then ascend up to the trunk then upper limbs and bulbar weakness. May start in the arms and move downward May begin in the arms and legs at the same time May occur in the nerves of the head only In mild cases, weakness or paralysis may not occur This weakness is symmytrical ( minor sides differences may occure), proximal and distal 9% is asymmetrical progress slowly over days or weeks Or abrupt and rapid

Clinical features Child becomes irritable . Parasthesia may occur, 89% pain accompany weakness 50% bulbar involvement . Facial nerve involved. also VI,III,XII,IX,X Some show viral meningitis or meningoencephalitis. Papillodema ( unexplained pathogenesis ) Respiratory muscules : reduced vital capacity CO2 retention even in absence of respiratory symptoms

Clinical features Features required for diagnosis Progressive motor weakness of more than one limb. Areflexia or hyporeflexia (loss of ankle jerks and diminished knee and biceps reflexes will suffice if other features are consistent with the diagnosis.

Clinical features Featrues supportive of diagnosis Progression :weakness may develop rapidly but cease to progress after 4wk . Roughly 50%will plateau within 2 wks , 80% by 3wks,and 90%by 4wks. Relative symmetry . Mild sensory symptoms and signs. Cranial nerve involvements like facial weakness develops in about 50% of patients. Autonomic dysfunction. Absence of fever at the onset of neurological symptoms. Recovery without specific therapy, begins 2-4wks after progression ceases, occasionally delayed for months.

Clinical features Features casting doubt on the diagnosis Marked persistent asymmetry in motor function. persistent bowel or bladder dysfunction at onset of symptoms . Discrete sensory level . Progressive phase longer than 4wks . CSF pleocytosis ( > 50wcc/mm3). Complete ophthalmoplegia (internal or external).

Clinical Phases Guillian-barre can be divided into five distinct clinical phases : Phase 1- first 24 hr from presentation Phase 2- disease progression Phase 3- plateau phase Phase 4- initial recovery Phase 5- rehabilitation

Dx Clinical CSF Electrophysiologic MRI

Investigation MRI of the brain and spinal cord Should be considered in all patients ,usually done if : The presentation is acute or rapidly progressive There are predominantly sensory symptoms (including back pain) There is predominant sphincter disturbance of presentation There is a clear sensory or marked motor level

Investigation Lumber puncture Elevated CSF protein without pleocytosis is a supportive diagnostic finding ,however the CSF may be normal within seven days of onset of symptoms Protein level :elevated (>45mg/dl ) after the first week of symptoms , peak 4-5 wks WCC <10/mm3 , occasionally up to 50 mm3 Glucose level normal

Investigation Neurophysiology Normal nerve conduction studies in the first week does not exclude the diagnosis of GBS In AIDP nerve conduction impairment = conduction block , decrease compound action potential amplitude

Investigation Since the median duration of excretion of Campylobacter in stools of infected persons is only 16 days and because of the 1- to 3-week lag time between infection and the onset of GBS, many GBS patients with preceding Campylobacter infection might have falsely negative stool cultures. multiple stool samples (or rectal swabs) should be obtained from GBS patients immediately upon admission to the hospital, preferably 3 over a 3-day period.

Investigation Other investigations Full blood count, blood culture ( if pyrexial ) Urea and electrolytes ( hypokalemia ) Creatine kinase (myositis) Chest x-ray , ECG Abdominal x-ray ( palpable bladder , constipation )

Treatment Admission IVIG Plasma exchange Supportive treatment Rehabilitation

treatment Symptomatic treatment is an essential part of the management of GBS. Children should admitted to the pediatric intensive care unit if they have one or more of the following : Flaccid tetraparesis Severe rapidly progressive course Reduced vital capacity at or below 20 ml/kg Bulbar palsy with symptoms Autonomic cardiovascular instability that is persistent hypertension or labile blood pressure or arrhythmias.

Plasma exchange Plasmapheresis has remained the gold standard treatment for GBS over the last 20 years. Should be used within 4 weeks of onset of neuropatic symptoms in non-ambulatory patients Should be used within 2 weeks of onset of neuropathic symptoms in ambulatory patients

treatment Plasmapheresis is generally safe in children who weigh 10 kg or more . A series of exchange with a cumulative total of approximately 250 ml/kg volume exchange or roughly a triple volume exchange . Disadvantages of Plasmapheresis include its rare complications, such as sepsis, risk of acquiring viral infections such as hepatitis and HIV.

treatment IVIG treatment has advantages over plasmapheresis because it is easier to administer, has significantly fewer complications, and is more comfortable for the patient. Side effects of IVIG expands the plasma volume so it must be administered with caution in patients with congestive heart failure and renal insufficiency fever, myalgia, headache, nausea, and vomiting, but these "influenza-like" symptoms are self-limiting. aseptic meningitis, neutropenia, and hypertension Anaphylaxis Thromboembolic events risk of serious hepatitis C infection transmission has been reduced

IVIG should be used within 2 weeks Corticosteroid not recommended Sequential treatment with PE then IVIG not recommended PE & IVIG recommended for the severe disease

treatment Pain management Pain of discomfort is present in 50-80%of children with GBS at the time of presentation .

treatment Managing pain by: Prevention of pain: Opioids Non steroidal anti-inflammatory drugs ( ibuprofen) Anti-epileptic drugs ( carbamazepine, gabapentine) Tricyclic antiderpessants (amitryptine) Prevention of pain: Air matresses Turning patients and carful positioning of limbs Continuation of enteral feeding ,effective antacids as omeprazole Preventing constipation Prevent and treat urinary retention.

treatment Supportive treatment directed to: Hypertension , hypotension Cardiac arrhythmia Pulmonary embolism (Prophylaxis for deep venous thrombosis should be provided because patients frequently are immobilized for many weeks). Bladder and bowel Psychological support Nutrition , fluid , electrolytes Pain Skin Cornea Joints Infection communication

prognosis 40% bed bound 15% require ventilation 90-95% complete recovery within 6-12months Remainder ambulatory with minor residual deficit 4% mortality rate Antecedents C.jujeni infection correlates with poor Px Causes of death : Autonomic (bradycardia , tachycardia , hypertension) Respiratory failure Pulmonary embolism Complication of ventilation Cardiovascular collapse

prognosis Chronic relapsing or chronic unremitting (7%) Features suggestive relapsing are: Severely weak Flaccid tetraplagia Bulbar and respiratory muscle involvement One or more relapses over 2mo.- years = CIDP Congenital GBS Weakness , areflexia , hypotonia . CSF and electrophysical studies suggestive of GBS . No treatment , gradual improvement.

Poliomyelitis

polioviruses RNA viruses, Picornaviridae family, enterovirus 3 genetically distinct serotypes Spread from intestinal tract to CNS 90 – 95 % inapperant infections Transmission: human is the only reservoir Fecal – oral route Isolated from stool for 2 weeks before paralysis to several weeks after onset of symptoms

Pathogenesis Wild type and vaccine strains Gain host entry through GIT Pass to the regional lymph nodes Goes to the blood causing viremia Wild type access the CNS through peripheral nerves Incubation period 8-12 days

Clinical manifestions Wild type follow one of the following courses : 90 -95 % inapparent infection (no disease & no sequelae 5% inabortive disease (influenza – like syndrome 1-2 wk after infection, fever, malaise, anorexia , headache +/- vomiting) then recovery complete

3- Non-paralytic poliomyelitis 1% Signs of abortive type, fleeting paralysis of bladder and constipation. This is first phase (minor) then symptoms-free period then major phase O/E: nuchal rigidity, changes in the deep and superficial reflexes (impending paralysis). No sensory defects

4- Paralytic poliomyelitis 0.1% Spinal type : major phase , sensory (paresthesia, hypersthesia), motor( fasiculation and spasms) progress to Asymmetric paralysis of one leg, then 1 arm DTR initially active then diminished and absent Variable course: some progress, some recover

5- Bulbar type +/- spinal cord involvement Nasal voice or cry Difficulty in swallowing Accumulated pharyngeal secretion Absence of effective coughing Nasal regurgitation Deviation of palate, uvula, tongue Involvement of vital centers in the medulla Paralysis of vocal cords … hoarseness, aphonia Sometimes culminate into ascending paralysis (Landry type)

6- polioencephalitis Rare Seizures , coma , spastic paralsysis , increased reflexes Respiratory insufficiency

7- Paralytic polio with respiratory insufficiency Anxious expression Inability to speak without frequent pauses Increased RR Movement of ala nasi, accessory muscles Inability to cough or sniff Paradoxical abdominal movement Relative immobility of intercostal space

Diagnosis Should be considered in any unimmunized or incompletely immunized child with paralytic disease Or any child with paralytic disease occurring 7-14 days after receiving the oral vaccine

Diagnosis Stool : Isolate the virus in 2 stool specimen collected with 24 – 48 hr apart Can isolate polio virus in 80 – 90 % in the first week and less than 20% within 3-4 wk CSF : normal in minor disease Cells 20 -200 / mm3 initially then reduced Protein : increase to reach 50 -100 mg/dl by 2nd week CSF serology : seroconversion or 4 folds rise in antibody titers

Treatment No specific treatment Supportive: Limit progression, prevent skeletal deformities, prepare child and family for prolonged treatment

Abortive poliomyelitis Analgesics, sedatives Attractive diet Bed rest until temperature normalize Avoidance of exertion for ensuing 2 wks Careful neurologic and musculoskeletal examination

Non paralytic poliomyelitis Same as abortive Relief muscle tightness Analgesics Hot packs for 15-30 min every 2-4 hr Hot tub baths Firm bed Footboard or splint to keep feet at right angle to legs Later gentle physical therapy

Paralytic poliomyelitis Hospitalization Physical rest Suitable body alignment to prevent deformity Change position every 3-6 hr Active and passive movement indicated as pain disappear Moist hot packs Opiates and sedatives Treat constipation Parasympathetic stimulant for bladder paralysis Adequate dietary and fluid intake Orthopedist, physiatrist should see them

Bulbar Maintain airway Avoid risk of inhalation Gravity drainage of accumulated saliva Nursed in lateral or semi-prone position Aspirators with rigid tips for oral pharyngeal secretion or flexible catheter for nasopharyngeal Fluid and electrolytes equilibrium Blood pressure taken at least twice Impaired ventilation signs should be noticed to decide tracheostomy

Prognosis Inapparent, abortive and aseptic meningitis = good outcome Paralytic = depends on the extent and severity of CNS involvement, recovery phase last 6 months Severe bulbar = MR 60% 30 – 40 % of persons survived paralytic polio… may experience muscle pain and exacerbation of exisiting weakness after 30 -40 yr

prevention Vaccination Hygienic mearures

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