Lecture 24- Acute Flaccid Paralysis PDF

Acute Flaccid Paralysis Dina Salama Abd Elmagid Assistant Professor of Pediatrics Mansoura University Children Hospital Faculty of Medicine, Mansoura University  ILOs Students will be able to: – Define and list the causes of acute flaccid paralysis...

Acute Flaccid Paralysis Dina Salama Abd Elmagid Assistant Professor of Pediatrics Mansoura University Children Hospital Faculty of Medicine, Mansoura University  ILOs Students will be able to: – Define and list the causes of acute flaccid paralysis – Know definition, clinical picture, investigations and treatment of Guillian Baree syndrome syndrome – Recognize transverse myelitis, myasthenia gravis and botulism  Definition Sudden onset of weakness or paralysis over a period of 15 days It’s a lower motor neuron lesion. LOWER MOTOR NEURON  Causes of AFP Spinal cord Acute transverse myelitis Anterior horn cell Polio virus and polio vaccination Peripheral nerve Guillian Baree syndrome (GBS) Toxic neuropathy (arsenic, lead) Neuromuscular junction Myasthenia gravis Botulism Muscle Inflammatory myopathies Critical illness myopathy  The Initial Complaint  Weakness is almost always first noted in the lower extremities  Abnormal gait is the initial complaint with proximal or distal leg weakness  Inquire about diplopia, drooping eyelids, difficulty chewing and swallowing, facial expressions, and voice changes  With proximal weakness  Difficulty ascending stairs with hip extensor weakness  Difficulty descending stairs with quadriceps weakness  Gower sign  With distal weakness  Stumbling is an early complaint  Falling on uneven surfaces at first, then “tripping on nothing at all  Clinical Approach Demonstrable motor weakness Clinical localization: spinal cord, peripheral nerve, NMJ, muscle DTR: absent / reduced/ normal Sphincters: preserved or affected Sensory loss: no loss, glove & stocking, or dermatomal.  Work up Accurate Clinical history is essential Muscle: Creatine kinase/ EMG/ Muscle biopsy Peripheral Nerve : - Unilateral : Nerve conduction study ( NCS), MRI lumbosacral - Bilateral : CSF examination, NCS Spinal Cord Lesion: - Urgent MRI spinal cord, CSF examination Neuromuscular junction: Repetitive nerve stimulation test (RNS)  Management 1. ABCs Ensure airway protected and adequate ventilation check BP / HR 2. Specific treatment Treatment of the cause Supportive Common Clinical Types of AFP Guillain Barre Syndrome ( GBS) The most common cause of acute flaccid paralysis Immune mediated disease GBS has been reported to follow : Vaccinations Epidural anesthesia Two third of cases present 2-4 weeks following Thrombolytic agents a respiratory or GI infection Campylobacter jejuni infection 20-30%, others (CMV, EBV, HSV)  Clinical features 1. Weakness: The classic clinical picture of weakness is ascending and symmetrical with areflexia, that ascends over hours to days to involve the arms, and in severe cases respiratory muscles 1. Cranial nerve involvement: 30% of the cases 2. Sensory changes: Paresthesia in the fingers and toes Pain is a common presentation (79%), particularly low back pain 3. Autonomic dysfunction: 50% of the cases, cardiac dysrhythmias, hypotension, hypertension, bladder dysfunction  The symptoms of the syndrome can be divided into three phases 1.The progressive phase, which lasts a few days to 4 weeks 2. The plateau phase, which consists of persistent symptoms and lasts for a few days or weeks 3. The improvement phase, when recovery takes place.  Workup 1) Nerve Conduction Studies: May be normal initially Essential to subtype GBS (axonal or demyelinating) 2) Cerebrospinal fluid analysis : (ALBUMINOCYTOLOGICAL DISSOSCIATION) Most, but not all, patients with GBS have an elevated CSF protein level (>50 mg/dL), with normal CSF cell counts 3) MRI spine: Non specific for diagnosis. However, it can reveal nerve root enhancement  How is Guillain-Barre syndrome usually treated? Treatment of GBS has two components: supportive care and specific therapy  Supportive care remains the cornerstone of therapy   General Specific  Preservation of ventilatory capacity  Intravenous immuneglobulin (IVIG)  Cardiovascular monitoring  Plasmapheresis  Treatment of autonomic disorders (arrhythmia, arterial hypertension)  Neurorehabilitation (motor stabilization, orthotics, splints)  Prevention and / or early treatment of infections  Adequate nutrition and hydration N.B The main modalities of therapy for GBS:  Preventing skin scars(breakdown) IVIG, Plasmapheresis  Treatment 0 IVIG Plasmapharesis may decrease Regimen the severity and shorten the duration of GBS Daily IVIG for 5 days at It is most beneficial within 7 a dose of 0.4 days, but still beneficial up to gm/kg/day, or 30 days after onset 2 gm/kg of IVIG given as a single dose or 1gm/kg/day for 2 days Glucocorticoids are not effective in GBS Need for intensive care : Children with any of the following problems should be admitted urgently to a pediatric intensive care unit :  Flaccid quadriparesis  Rapidly progressive weakness  Reduced vital capacity (≤20 mL/kg)  Bulbar palsy  Significant autonomic instability  Transverse Myelitis  Acute demyelinating disorder of the spinal cord (other parts of CNS can affected)  Commonly preceded by a viral infection or immunization  Symmetrical leg weakness, and early bladder involvement Back pain is common at the onset  Paraplegia with areflexia but later hyperreflexia Exclusion of acute cord  Sensory level of loss of sensation compression is essential  Treatment: IV Solumedrol  Myasthenia Gravis  Autoimmune disease  Acetylcholine receptor antibody positive in 85% of cases  Weakness and fatigability of muscles (> 50% ocular weakness, 15% bulbar weakness, < 5% limb weakness alone)  Involvement of respiratory muscles is a serious life-threatening condition termed myasthenic crisis.  Botulism  Acute descending paralysis  Blurred vision, diplopia, ptosis, dysarthria, dysphagia  Autonomic: constipation, dry mouth, urine retention, postural hypotension  No fever, consciousness not impaired