Acute Flaccid Paralysis Lecture Notes PDF

Hamza alrawajfah +-----------------------------------+-----------------------------------+ | **Muscular Dystrophy** | **Myasthenia Gravis A | | | pediatrician examines an infant | | **Duchenne A 3-year-old boy is | with poor**...

Hamza alrawajfah +-----------------------------------+-----------------------------------+ | **Muscular Dystrophy** | **Myasthenia Gravis A | | | pediatrician examines an infant | | **Duchenne A 3-year-old boy is | with poor** | | brought to the pediatrician | | | because he is very clumsy.** | **sucking and** | | | | | **According to his parents, he | **swallowing since birth. The | | has difficulty climbing stairs | infant is** | | and frequently falls. On physical | | | examination hypertrophy of the | **noted to be a floppy baby with | | calves is noted. Primary | poor head control. There is** | | myopathy with genetic basis; is | | | progressive and results in | **associated ocular ptosis and | | degeneration and death of muscle | weak muscles on repeated use. | | fibers; most common of the | Immune- mediated neuronal | | neuromuscular** | blockade; motor end plate is | | | less** | | **diseases in all races and | | | ethnic groups; X-linked recessive | **responsive due to, decreased | | Clinical** | number of available** | | | | | **presentation − First sign may | **acetylcholine receptors | | be poor head control in infancy. | secondary to circulating receptor | | − By year 2, may have subtle | binding antibodies;** | | findings of hip- girdle weakness | | | − Gower sign as early as age 3 | **generally nonhereditary | | years but fully developed by age | Clinical presentation − Ptosis | | 5--6 years; with hip waddle gait | and extraocular muscle weakness | | and lordotic posturing − Calf | is the earliest and most | | pseudohypertrophy (fat and | consistent finding.** | | collagen) and wasting of** | | | | | | **thigh muscle** | | +===================================+===================================+ | | | +-----------------------------------+-----------------------------------+ Acute Flaccid Paralysis(AFP) report disease like chicken box اذا صار عندي epidemic  - **Sudden onset of weakness or paralysis. Weakness is a decreased ability to voluntarily move muscles. An emergency in which management priorities are to :** - **support vital functions** - **reach a specific diagnosis Anterior Horn Cell(AHC):** - **Acute Poliomyelitis** - **Acute transverse myelitis Peripheral nerves:** 1. **GBS** - **Botulinum toxin Metabolic :** - **Periodic paralysis Muscular :** - **Myositis** - **Guillain-BarréSyndrome(GBS)** - **Poliomyelitis.** - **Transverse Myelitis. Guillain-Barré Syndrome(GBS)** - **The most common cause of acute flaccid paralysis in healthy infants and children.** - **Post- infectious polyneuropathy مريض كان مرة اخر متى الاهل نسال** - **An acute , rapidly progressing and potentially fatal.** - **Immune mediated disease.**- **- There is no known genetic factors.** two third of cases follow a respiratory or GI infection. - **Demyelination of nerve sheath due to autoimmune disorder** Inflammation causes leakage of proteins into the CSF causing raised CSF proteins without pleocystosis can involve the peripheral nerves, cranial nerves , dorsal roots, dorsal ganglia & sympathatic chain. - **Campylobacter(gastroenteritis) infection is the most common, but other organisms include CMV, EBV, HSV, Enteroviruses,...** - **The classic presentation:** - **Weakness begins in LL ( The characteristic symptoms and signs tried are areflexia, flaccidity,** - **Progressively involve trunk,UL & finally bulbar muscles. Tongue pharynx larynx** - **Proximal & distal muscles are involved relatively symmetrically** - **Onset is gradual & progress over days or weeks.** - **Affected children are irritable** - **Weakness may progress to inability or refusal to walk** - **parasthesia occur in some cases** - **Bulbar involvement occur in about half of cases.** - **Respiratory insufficiency may result** - **Dysphagia and facial weakness are often impending signs of respiratory failure. By the peak of the illness:** - **46% had cranial nerve involvement - 24% could not use their arms** - **13% required mechanical ventilation could be vary from mild to sever** - **\>90% of patients reach the nadir of their function within two to four weeks** - **return of function occurring slowly over the course of weeks to months.** - **The clinical course of GBS in children is shorter than in adults and recovery is more complete. GBS IN CHILD SHORT AND GOOD RECOVERY THAN ADULT** - **Cerebrospinal Fluid:** \- After the first week of symptoms typically reveals:IF YOU DO THIS FINDIG WITHIN FIRST DAY This finding don't reflect any pathology you need at least one weak - **Normal pressures** - **Normal cell count** - **Elevated proteins (greater than 50 mg/dL)** - **Early in the course (less than one week), protein levels may not yet be elevated.** - **Electrophysiologic studies(Electromyography, Nerve conduction studies)** - **Most specific and sensitive tests for diagnosis** - **Evidence evolving multifocal demyelination** - **A normal study after several days of symptoms, makes the diagnosis of Guillain-Barré syndrome unlikely.** - **Marked persistent asymmetry of weakness?.** -**Persistent bladder or bowel dysfunction.** Could be spinal cord injure or tumor -**Bladder or bowel dysfunction at the onset.** -**Sharp sensory level.** - **20% of children with GBS require mechanical ventilation for respiratory failure. Its serious condition** - **Intravenous Immunoglobulins if pt don't response you use plasmapheresis** - **Plasmapheresis** to ger rid AB Corticosteroids are not effective and not indicated Prognosis despite - **In general, the prognosis in affected children is better than adults. If you suspect pt with AFP you must admit to ICE BCZ continuous monitoring vital sign bcz its progress in some case and slow in some case** - **Recurrences are uncommon but can occur in children. But could be happen** - **At long-term follow up, 93% were free of symptoms, and the remainder were able to walk unaided. Some they have it residual deficit** - **Mortality is approximately 3 to 4%, and usually is secondary to autonomic dysfunction and respiratory failure.** - - **presumed autoimmune mediated inflammation and demyelination of the spinal cord.** - **Post-infectious etiology largely predominates in children** - **Mean age of onset is 9 years.** - **Symptoms progress rapidly, peaking within 2 days.** - **Usually level of myelitis is thoracic.** - **Asymmetrical leg weakness, sensory level and early bladder involvement.if tou think ATM you must requesr urdent spinal MRI** - **Recovery usually begins after a week of onset. Diagnosis depend on Physical Examination** - **Funduscopic examination for optic neuritis.** - **Increased tone, spastic weakness, legs more than arms hypertonia bcz its upper motor lesion** - **Reflexes are sealing in spinusually brisk, with positive Babinski sign.** - **sensory level.** - **Sphincter dysfunction.** - **CSF analysis &MRI brain and spine are the two most important tests and are mandatory. Lumbar Puncture shows:** - **Normal or slightly increased protein.** - **Mild pleocytosis with lymphocyte predominance. Treatment of ATM** - **IV methylprednisolone may be useful in ATM** - **IV immunoglobulin (IVIG) or plasmapheresis may be a safe and effective therapeutic alternative in patients that do not respond to or intolerant of IV methylprednisolone.** - **Symptomatic management of bowel and bladder dysfunction.** - **Management of respiratory, cardiovascular & autonomic dysfunction.** - **Physical and occupational therapy (PT/OT) may help promote functional recovery and prevent contractures** - **50% make a full recovery within 3 to 6 months.** - **40% recover incompletely.** - **10% don't recover.** - **Incubation period of 7 to 14.** - **Transmitted by oral-fecal contact. Most common bcz its enterenterovirus** - **Person-to-person spread is the most common means of transmission, followed by contaminated water.** - **During epidemics, it also may be transmitted by pharyngeal spread.** - **Poliovirus initially infects the GI tract. It may spread to lymph nodes and rarely to CNS.** - **The mechanism of spread of poliovirus to the CNS is not well understood. Poliomyelitis: Risk Factors** - **Immune deficiency** - **Pregnancy** - **Poor sanitation and hygiene** - **Poverty** - Unimmunized status, especially if \ - **Based on the clinical presentation.** - **Cerebrospinal Fluid: viral meningitis** - **Polymerase chain reaction amplification of poliovirus RNA from CSF or serologically, by comparing viral titers in acute and convalescent sera** - **Virus recovery from stool, throat washing, blood.** - **Virus recovery from stool is essential to diagnosis.** - **Obtain stool, blood and throat samples for viral serology, demonstrating a four fold rise in IgG is helpful but not always easy.** - **Positive IgM is diagnostic. Diagnosis...cont** - **Electro-diagnostic investigations reveal normal sensory nerve studies.** - **Motor nerve studies:** - **MRI may be helpful to evaluate involvement of anterior horn of the spinal cord or other findings. Treatment** - **No definitive treatment. No found antivirus treatment again poliovirus** - **Mainly supportive: pain relief and physical therapy for muscle spasms.** - **Mechanical ventilation: Respiratory failure. Clinical Course & Outcome** - **About two-thirds of patients with acute flaccid paralysis do not regain full strength. Especially old age** - **The more severe the acute weakness, the greater the chance of residual deficits.** - **The mortality was 5 to 10% in the era of epidemics, and approached 50% for those with bulbar involvement because of cardiovascular and respiratory complications. Most common in poliomyelitis than GBS AND ATM**