Lec 18 Acute Flaccid Paralysis - Dr. Mahdi PDF

Hamza alrawajfah  Acute Flaccid Paralysis ) What indicator it is tumor ? it was focal...

Hamza alrawajfah  Acute Flaccid Paralysis ) What indicator it is tumor ? it was focal meningitis you must do ‫اذا بشك انه عند المريض‬ Nurophysical exam Assessment tone in infant have extra maneuver like vertical and ventral suspensionhttpshttps://youtu.be/lk9YJYwO6 VQ?si=EPcCwm3SKmZyxQPx Head lag&gower sign Muscular Dystrophy Myasthenia Gravis A Duchenne A 3-year-old boy pediatrician examines is brought to the pediatrician an infant with poor because he is very clumsy. sucking and swallowing since According to his parents, he birth. The infant is has difficulty climbing stairs noted to be a floppy and frequently falls. On baby with poor head physical examination control. There is hypertrophy of the calves is associated ocular noted. Primary myopathy ptosis and weak with genetic basis; is muscles on repeated progressive and results in use. Immune- degeneration and death of mediated neuronal muscle fibers; most common blockade; motor end plate is less of the neuromuscular responsive due to, diseases in all races and decreased number of ethnic groups; X-linked available recessive Clinical acetylcholine presentation − First sign may receptors secondary be poor head control in to circulating receptor infancy. − By year 2, may binding antibodies; have subtle findings of hip- generally girdle weakness − Gower nonhereditary sign as early as age 3 years Clinical presentation − Ptosis and extraocular but fully developed by age muscle weakness is 5–6 years; with hip waddle the earliest and most gait and lordotic posturing − consistent finding. Calf pseudohypertrophy (fat and collagen) and wasting of thigh muscle  1  Spinal Muscle Atrophy (SMA) A pediatrician examines an infant who is on the examination table in frog-leg position, with subdiaphragmatic retractions and absent tendon reflexes. Degenerative disease of motor units beginning in the fetus and progressing into infancy; denervation of muscle and atrophy Clinical presentation—SMA 1 presents in early infancy with − Progressive hypotonia; generalized weakness; Infant is flaccid, has little move ment and poor head control − Feeding difficulty − Respiratory insufficiency − Fasciculations of the tongue and fingers − Absent DTR  Acute Flaccid Paralysis(AFP) report disease like chicken box epidemic ‫ اذا صار عندي‬number ‫مهم حتى اصير عندي‬Report  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 ‫االهل رح يجو يحكو انه الطفل ما بقدر انه يمشي‬ - reach a specific diagnosis ‫ اذا في‬physical exam ‫ وببلش‬history ‫بمشي لتا‬ ‫ اذا مشكلة بالمفصل‬AFP‫ بفكر‬neurological manifastration Anterior Horn Cell(AHC): myositis ‫ اذا مشكل بالعضالت بفكر‬RA ‫بفكر‬  Acute Poliomyelitis  Acute transverse myelitis Peripheral nerves: 1. GBS Toxins (Diphteria)when pt don’t have vaccination Neuromuscular junction:  Botulinum toxin Metabolic :  Periodic paralysis ‫ بعدين‬weakness ‫ بيجي‬Family history the problem in K CHANNAL Muscular : recover after k supplement  Myositis Acute Flaccid Paralysis ‫ بتروح‬GBS ‫ وما فكرت‬weakness ‫اذا اامريض مش عارف يمشي او عنده‬  Guillain-BarréSyndrome(GBS) arrythmia ‫ و‬cardic or rs failure ‫المريض وبجيك ثاني يوم ب‬  Poliomyelitis. diagnosis not initiation evaluation ‫االعتماد على‬  Transverse Myelitis. ‫ الزم ناخذة بجدية‬complain weakness ‫اي‬ Guillain-Barré Syndrome(GBS)  The most common cause of acute flaccid paralysis in healthy infants and children. 2   Post- infectious polyneuropathy ‫نسال االهل متى اخر مرة كان مريض‬  An acute , rapidly progressing and potentially fatal. GBS pathophysiology  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,… Clinical presentation ascending or not ‫بالبداية مارح اعرف اذا‬ symmetrical or not ‫بس رح اعرف اذا‬ Usually 2 - 4 weeks following respiratory or GI infection.  The classic presentation:  Weakness begins in LL ( The characteristic symptoms and signs tried are areflexia, flaccidity, and symmetrical ascending weakness.)  Progressively involve trunk,UL & finally bulbar muscles. Tongue pharynx larynx  Proximal & distal muscles are involved relatively symmetrically not just hours could be day ‫ مش‬Acute Clinical presentation or two day  Onset is gradual & progress over days or weeks. ‫ بقعد اشهر حتى تبين‬chronic ‫بالمقابل‬  Affected children are irritable presentation  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: - 79% had neuropathic pain - 60% could not walk - 51% had autonomic dysfunction - 46% had cranial nerve involvement - 24% could not use their arms - 13% required mechanical ventilation could be vary from mild to sever 3 Physical examination Symmetric limb weakness -diminished or absent reflexes -Vibration and position sensation are affected in 40% of cases. Autonomic dysfunction: Cardiac dysrhythmias. Orthostatic hypotension, hypertension mostly serious Paralytic ileus, Bladder dysfunction Clinical course /GBS  >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 Diagnosis of GBS  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 ‫ هون‬PROCESS infection ‫ بفكر‬increase WBC ‫اذا كان‬ immunity process  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. Diagnosis of GBS  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. Doubt the Diagnosis of GBS IF  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. GBS Management  20% of children with GBS require mechanical ventilation for respiratory failure. Its serious condition Special Therapy Immune modulatory therapy:  Intravenous Immunoglobulins if pt don’t response you use plasmapheresis 4   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. spinal ‫ على‬change in sympathetic ‫ ما بنعرف متى رح اصير‬normal ‫ بالبداية بتكون‬Vital sign  cord ‫ النه‬hours 2-1 vital sign ‫ بحتاج اخذ‬change ‫ ساعات اذا صار‬6-4 ‫ كل‬vital sign ‫ باخذ‬floor ‫اذا حطيتخ بال‬ arrythmia ‫ممكن اصير عندهم‬ initiation not sever you ‫ حتى اذا كان‬ICE ‫ الزم ادخل المريض‬AFP ‫ اذا بدي افكر‬Important  need to admit he to observation  Acute transverse myelitis (ATM): Inflammation of the spinal cord causing acute/or subacute loss of motor, sensory and autonomic function, often evolves in hours or days. Pathophysiology  presumed autoimmune mediated inflammation and demyelination of the spinal cord.  Post-infectious etiology largely predominates in children GBS demyelination in pn Transverse myelitis ATM demyelination anterior horn  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. 5  swealing in spinal cord indication inflammatory condition ‫بكون في‬ hematoma ‫ او‬hemorrhage ‫ اتاكد مافي‬exclude other disorder or disease  sensory level. ‫ اذا كانو عللى‬presentation ‫ النهم بيجو بنفس‬compress on spinal cord ‫ عمل‬tumor‫او‬  Sphincter dysfunction. level ‫نفس‬ Diagnosis  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. Treatment of ATM  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 Prognosis  50% make a full recovery within 3 to 6 months.  40% recover incompletely.  10% don’t recover. Poliomyelitis: Etiology: Caused by a poliovirus. 3 serotypes of poliovirus Type 1 most frequently associated with epidemics. Poliomyelitis In 1% of cases virus invades CNS.mostly gastroenteritis not cns Multiplies and destroys anterior horn cells. In severe cases, poliovirus may attacks motor neurones in brainstem, leading to difficulty in swallowing,speaking and breathing increase mortality rate ‫اذا وصل لهاي المرحلة‬ 6 Poliovirus: Pathogenesis  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. Poliovirus: Pathogenesis  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