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Approach to a child with weakness

Approach to a child with weakness . Dr. Pushpa Raj Sharma Professor of Child Health Institute of Medicine. Upper Motor Neuron. Lower Motor Neuron Myopathic. Weakness/paresis/paralysis. Pattern of weakness. Sign UMN LMN Myopathic Atrophy - +++ + Fasciculations - +++ -

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Approach to a child with weakness

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  1. Approach to a child with weakness Dr. Pushpa Raj Sharma Professor of Child Health Institute of Medicine

  2. Upper Motor Neuron. Lower Motor Neuron Myopathic Weakness/paresis/paralysis

  3. Pattern of weakness Sign UMN LMN Myopathic • Atrophy - +++ + • Fasciculations - +++ - • Tone +++ - +/- • Distribution Regional Segmental Proximal • Tendon reflexes +++ -/-- +/- • Babinski’s sign + - -

  4. Important fact: • When there is a discrepancy between the history and physical findings, it is usually because the patient complains of weakness, whereas symptoms are actually due to other causes. • Child Abuse may be the cause.

  5. Motor Neuron Disease • Asymmetric • Upper and lower motor neurones • Amyotrophic Lateral Sclerosis (ALS) • Sporadic ALS • Hereditary ALS • Superoxide Dismutase; Chromosome 21; Dominant • Other ALS: Recessive & Dominant • Multi-system disorders • ALS with Ophthalmoplegia & Extrapyramidal Disorders • Polyglucosan body disease • Motor neuronopathy with cataracts and skeletal abnormalities • Multiple system atrophy

  6. Motor Neuron Disease • Symmetrical and proximal • SMN: Chromosome 5q; Recessive • Androgen Receptor (Bulbo-spinal Muscular Atrophy): X-linked; Recessive • Hexosaminidase A (Tay-Sachs): Chromosome 15; Recessive • Hand weakness • Bulbar involvement

  7. Motor Neuron Disease • Lower motor neurones only • Distal Lower Motor Neuron (LMN) Syndrome • IgM vs GM1 ganglioside • IgM vs GalNAc-GD1a ganglioside • Also see: Multifocal motor neuropathy • Proximal Lower Motor Neuron Syndromes • Brachial amyotrophic diplegia • ? Associated with IgM vs asialo-GM1 • Rare: Upper > Lower limbs with anti-Hu antibodies

  8. Motor Neuron Disease • Lower Motor Neuron Syndrome without antibodies (PMA) • ALS variants • Hereditary • Sporadic • Focal motor neuron disease • Monomelic Amyotrophy • Paraspinous muscle amyotrophy • Cervical amyotrophy

  9. Motor Neuron Disease • Paraneoplastic motor neuro(no)pathy • Mild weakness: With lymphoma • Severe weakness: With breast cancer • Hopkins' syndrome: Acute post-asthmatic amyotrophy • Polio & Post-polio syndrome • SMN2 (SMNC) deletions • Neurofibromatosis, Type 25

  10. Motor Neuron Disease • Symmetric & Proximal: Hereditary Spinal Muscular Atrophy • SMN: Chromosome 5q; Recessive • Androgen Receptor (Bulbo-spinal Muscular Atrophy): X-linked; Recessive • Hexosaminidase A (Tay-Sachs): Chromosome 15; Recessive

  11. Motor Neuron Disease • Rapid onset • Acute Axonal Motor Neuropathy • (with Campylobacter jejuni or serum IgG vs GM1) • Poliomyelitis • Porphyria • 4 types cause neurologic attacks • Acute intermittent • Variegate Porphyria • Coproporphyria • δ-amino-levulinic acid dehydratase deficiency • Urine: All types produce increased δ-amino-levulinic acid during attacks

  12. Motor Neuron Disease • Painful • Acquired • Others

  13. Motor Neuron Disease • Acquired • Toxic: Lead; Dapsone; Botulism; Tick Paralysis • Infections • Polio • West Nile • Central European encephalitis • Creutzfeld-Jacob • Amyotrophy • Polyneuropathy (± Demyelinating)

  14. Weakness/ paresis/ paralysis indicates the lesions in the upper motor nurone or lower motor neurone or myoneural junction or muscles. Preservation of sensation/ Increased or absent or diminished jerks/ atrophy or hypertrophy indicates the site of lesions.

  15. The major clinical diagnosis associated with AFP (n= 517) * • Guillain-Barre syndrome (30.2%), • Central nervous system infection (16.2%), • Transverse myelitis (10.6%) • Non-polio enterovirus infection (6.2%), • Hypokalaemic paralysis (5.2%). * Hussain IH, Ali S, Sinniah M, Kurup D, Khoo TB, Thomas TG, Apandi M, Taha AM J Paediatr Child Health. 2004 Mar;40(3):127-30

  16. Age distribution of Guillain-Barré syndrome. AIDP=acute inflammatory demyelinating polyradiculoneuropathy; FS=Fisher syndrome Lyu, R.-K. et al. J Neurol Neurosurg Psychiatry 1997;63:494-500

  17. Seasonal distribution of Guillain-Barré syndrome Lyu, R.-K. et al. J Neurol Neurosurg Psychiatry 1997;63:494-500

  18. Recurrent Guillain Barre' Syndrome • Of the 220 patients of acute idiopathic demyelinating polyneuritis (AIDP/GBS) seen over a seven year period, 15 patients (M:F:11:4) had a relapsing course (6.8%). They had 36 episodes at a variable interval of 3 months to 25 yrs. • Taly AB, Gupta SK, Anisya V, Shankar SK, Rao S, Das KB, Nagaraja D, Swamy HS.J Assoc Physicians India. 1995 Apr;43(4):249-52.

  19. Follow-up of AGBS • At a follow-up of 1 year or more, 20 patients recovered and 3 had residua. • Hung PL, Chang WN, Huang LT, Huang SC, Chang YC, Chang CJ, Chang CS, Wang KW, Cheng BC, Chang HW, Lu CH. Pediatr Neurol. 2004 Feb;30(2):86-91. • permanent neurological defects in children under 15 years of age was 1.4/10 million annually • Rantala H, Uhari M, Niemela M. Arch Dis Child. 1991 Jun;66(6):706-8; discussion 708-9.

  20. Subacute Inflammatory Demyelinating Polyneuropathy. • progressive motor and/or sensory dysfunction consistent with neuropathy in more than one limb with time to nadir between 4 and 8 weeks, • electrophysiologic evidence of demyelination in at least two nerves, • no other etiology of neuropathy, and • no relapse on adequate follow-up. Complete recovery was achieved in 69% of cases and partial recovery in others. • Oh SJ, Kurokawa K, de Almeida DF, Ryan HF Jr, Claussen GC.Neurology. 2003 Dec 9;61(11):1507-12.

  21. Chronic Inflammatory Demyelinating Polyradiculoneuropathy • A low frequency of antecedent events. • Weakness accompanied by sensory loss and diminished tendon reflexes • Pain and cranial neuropathies are uncommon • Progressive weakness for two months. • Sladky JT; Ashwal S: Peripheral neuropathies in childre. Pediatric Neurology Principles and Practice. Ed. Swaiman KS; Ashwal S. 3rd ed.1999. Publisher: Mosby

  22. Causes Inflammatory Polyradiculoneuropathy • GB Syndrome 58% • Chronic inflammatory demyelinating polyneuropathy 31% • Associated with collagen vascular disease 7% • Other immune/infectous disorders 4%

  23. HOPKINS' SYNDROME: Acute post-asthmatic amyotrophy • Onset • After acute asthmatic attack: Latency 1 to 18 days • Mild pain: Limb, neck or meningismus • Rapid onset weakness

  24. HOPKINS' SYNDROME: Acute post-asthmatic amyotrophy • Weakness • Single limb; Asymmetric; May be Proximal > Distal • Severity: Mild to severe • Arm or leg • Sensory: Normal

  25. HOPKINS' SYNDROME: Acute post-asthmatic amyotrophy • CSF • Pleocytosis • Protein: ± Increased • MRI: May show signal (T2) in spinal cord • Prognosis: Permanent paralysis

  26. Flaccid or hyper tonicity

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