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Common causes of childhood paralysis Behaylu Yibe – Pediatrics Neurologist Definition Acute flaccid paralysis (AFP) is an acute onset flaccid weakness, less than 4 weeks, reaching its maximum severity in days to weeks. Acute flaccid paralysis is a complex clinical syndrome that requires i...

Common causes of childhood paralysis Behaylu Yibe – Pediatrics Neurologist Definition Acute flaccid paralysis (AFP) is an acute onset flaccid weakness, less than 4 weeks, reaching its maximum severity in days to weeks. Acute flaccid paralysis is a complex clinical syndrome that requires immediate and careful evaluation for making a diagnoses Some of the causes of AFP are GuillainBarre syndrome (GBS), poliomyelitis, transverse myelitis (TM), traumatic neuritis and post di Acute Flaccid paralysis Localization Differential diagnosis Brain stem: GBS with cranial nerve involvement, brain stem encephalitis and stroke Spinal cord: Acute transverse myelitis, acute myelopathy due to spinal cord compression (abscess, space occupying lesion), anterior spinal artery syndrome. Anterior horn cells: Poliomyelitis, non-polio enteroviruses. Nerve root (radiculopathy): Guillain Barre syndrome. Peripheral nerve: Guillain Barre syndrome, toxic neuropathies (diphtheria, tick bite paralysis, lead, arsenic poisoning) traumatic neuritis, acute intermittent porphyria, critical illness neuropathy. Neuromuscular junction: Myasthenia gravis, botulism, snake bite, organophosphorus poisoning Muscle: Polymyositis, hypokalemia, hypophosphatemia. Clinical approach Usually sudden occurrence of flaccid weakness denotes lower motor neuron (LMN) weakness. However, upper motor neuron (UMN) weakness of sudden onset also can have flaccid weakness in the initial stages due to the neuronal shock state Following a systematic clinical approach based on the distribution and progression of weakness, associated sensory involvement, fever etc. will help in differentiating various conditions. Where is the lesion Is it neurologic condition Next step in the diagnosis is localizing the lesion; based on clinical signs. LMN: Flaccid weakness with absent reflex is in favor of LMN Exact site of lesion is determined by verifying the presence or absence of cranial nerve lesions, bladder involvement, tendon reflexes and sensory involvement. Anterior horn cell disorders Seven year old child was seen with history of fever, severe myalgia, back pain and weakness of abduction of right shoulder. Examination revealed meningeal signs, mild bulbar weakness, normal sensory and sparing of bladder and bowel. CSF showed lymphocytic pleocytosis. NCV/EMG suggested anterior horn cell disease. Serology revealed enterovirus (type was not determined) on follow up flail shoulder with severe wasting of deltoid was persisting Poliomyelitis Febrile illness, rapidly progressive asymmetric weakness, preserved sensory function, severe myalgia and residual paralysis after 60 days are features of polio like illness. Enterovirus 71, Coxsackie, Echo and West Nile viruses are the non polio viruses reported. Poliomyelitis According to AFP surveillance, two stool samples must be collected 24 to 48 hours apart in the first 14 days following the onset of paralysis and to be sent to the accredited lab after following the surveillance protocol for polio or related viral isolation. Poliomyelitis The patients with AFP within the last 6 months should be reported to the surveillance Medical Officer of WHO. The four steps of AFP surveillance are finding and reporting children with AFP, transport and analysis of stool sample, identify poliovirus in laboratory and determine the virus strain and origin. Poliomyelitis The non-polio AFP rate is an indicator of surveillance sensitivity and should be equal to or more than 1: 100,000 (background rate of AFP) according to National Polio Surveillance Project AFP surveillance is for detecting polio virus transmission Outbreak response immunization (ORI) The incubation period of the polio virus is 4-35 days prior to weakness and all children 0-59 months of age in the affected area (around 500 children) where the child resided or visited in the incubation period are given active immunization. Poliomyelitis The trivalent OPV which was in use till 2016 had a highest sero conversion rates for type 2 and hence wild polio virus type 2 was eradicated in 1999. Most cases of vaccine derived Polio Myelitis are due to OPV 2 and the trivalent OPV was withdrawn and replaced with bivalent OPV since April 2016. Radicles and peripheral nerve Ten year old boy was admitted with weakness of lower limb of five days and upper limb of two days duration. He had difficulty sipping water from a glass was unable to wear slippers and hold the objects in hand. On 3rd day as he developed difficulty of using his hands and respiratory muscle weakness. He had pain in the legs but no sensory loss. There was no difficulty in urination. He had an upper respiratory tract infection 2 weeks ago. Guillain Barre syndrome (GBS) Pure proximal motor limb weakness with bifacial palsy and presence of respiratory muscle weakness is diagnostic of GBS. GBS presenting as painful limping may be confused as synovitis. Absent reflexes points towards GBS than synovitis. Bifacial palsy can be missed as there is no facial deviation due to the symmetric weakness. Difficulty puckering the lips or sipping, incomplete burying of eye lashes on tight eye closure are the clues. Guillain Barre syndrome (GBS) It is a progressive, near symmetrical weakness occurring in more than one limb with areflexia GBS GBS Diagnosis of GBs Electrophysiological abnormalities of GBS: Nerve conduction studies (NCV) are helpful in confirming the diagnosis. The first changes in AIDP are delayed or absent F and H responses, reflecting proximal demyelination. Prolonged distal latencies, decreased conduction velocities along with evidence of segmental demyelination, (conduction block and temporal dispersion) are other changes present in 50% of patients by 2 weeks and in 85% by 3 weeks. GBS IVIG 400mg/kg/day for 5days in the presence of rapidly progressive weakness (Modified Hughes GBS disability scale), presence of respiratory or bulbar involvement. Ventilatory support may be required when there is respiratory failure. IVIG is not indicated if the degree of weakness is non-progressive and has been present for more than 4weeks. GBS Acute transverse myelitis (TM) It presents as sudden limb weakness, bowel-bladder and sensory disturbances and commonly occurs in toddlers and adolescents. Postinfectious TM: Preceded by viral or bacterial infection. Viruses implicated are enteroviruses, coronavirus, coxsackie, cytomegalovirus, Epstein-Barr, herpes-simplex. Bacteriae include mycoplasma pneumoniae, rickettsia, beta hemolytic Streptococcus, borrelia, chlamydia and leptospira. Post-vaccinal TM: May follow immunization for rabies, hepatitis B, influenza, Japanese encephalitis, diphtheria/ pertussis/tetanus, measles, mumps, rubella, pneumococcus, polio, smallpox and varicella. Diagnosis Acute flaccid paralysis with sensory level is a feature of TM. In children, mild sensory symptom like hyperaesthetic sites may be present which helps for localization. If legs are affected the sensory level is commonly at umbilicus or at the level of nipple. If the arms are weak, look for sensory level at cervical region. Bowel and bladder involvement cause constipation and urinary retention. Respiratory failure (intercostals or diaphragmatic weakness) may occur with higher level lesions. Diagnosis TM Intravenous methyl prednisolone (30 mg/kg up to 1000 mg daily) for five days. Plasma exchange or, intravenous cyclophosphamide (800 to 1200 mg/m2 administered as a single pulse dose. Diagnosis Variants Variants of TM: Identifying TM variants will help in etiological diagnoses and thus treatment and prognoses. Longitudinally extensive TM (LETM) - Lesion in >3 spinal segments associated with bilateral optic neuritis = neuromyelitis optica spectrum disorder. Acute flaccid myelitis (AFM) - Like poliomyelitis they have segmental LMN (AHC) and additional UMN lesion in the setting of other viral infections - e.g. enterovirus. These patients may recover with residual wasting and weakness. Acute partial TM - Asymmetric extending one to two spinal segments Compressive myelopathy can present as acute syndrome. Traumatic neuropathy (TN) Traumatic neuropathy (TN) It includes injury to plexus, roots or peripheral nerves. Result from penetrating trauma (injections, falling on sharp objects), entrapment or from traction injuries. Focal weakness is attributable to a single or multiple nerve distribution. The common cause is an injection to gluteal or deltoid region The knowledge of anatomy of nerve will help in identifying the specific nerve. The diagnosis is confirmed by nerve conduction studies. The prognosis depends on severity of injury. Persistent, severe and refractory neuropathic pain may be present along with weakness. Muscle Eleven year old girl was admitted with 2 weeks history of painful weakness of upper and lower limbs. No sensory signs. Examination showed grade 3 power in proximal and grade 4 in distal muscles. She had neck flexor weakness. Knee jerk and biceps jerk were very sluggish with easily elicitable ankle jerk. Skin examination showen. CPK was found be raised. Polymyositis-dermatomyositis AFP can be due to myositis also. Polymyositisdermatomyositis can be suspected when there is a symmetric proximal muscle weakness without sensory symptoms or signs and with preserved reflexes, neck muscle weakness, muscle pain and raised CPK. Acute myositis following viral infection also are not uncommon. Temporal relation with viral illness, high CPK and rapid improvement are the features. Neuromuscular junction disorders Neuromuscular junction disorders A girl aged nine years was admitted in ICU with rapidly worsening limb weakness and severe respiratory paralysis who required ventilation for 5 days. This happened after a bout of urinary tract infection, which was treated with ciprofloxacin. History of ptosis for 2 weeks occurring in evening hours. The diagnosis of myasthenic crisis. Rapid deterioration was due to the usage of ciprofloxacin. She improved with IVIg and neostigmine treatment. Acetylcholine receptor (AChR) antibody was positive. localization Thank YOU