Approach to a child with weakness by sid76703

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									Approach to a child with
      Dr. Pushpa Raj Sharma
     Professor of Child Health
          Institute of Medicine
                   Upper Motor Neuron.

                   Lower Motor Neuron

                   Myopathic
   Pattern of weakness

  Sign              UMN        LMN         Myopathic
 Atrophy           -          +++           +

 Fasciculations    -          +++           -

 Tone              +++        -             +/-

 Distribution      Regional   Segmental     Proximal

 Tendon reflexes   +++        -/--          +/-

 Babinski’s sign   +          -             -
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.
Motor Neuron Disease
  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
           Polyglucosan body disease
           Motor neuronopathy with cataracts and skeletal
           Multiple system atrophy
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;
Hand weakness
Bulbar involvement
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
Motor Neuron Disease
   Lower Motor Neuron Syndrome without antibodies
   ALS variants
      • Hereditary
      • Sporadic
   Focal motor neuron disease
      • Monomelic Amyotrophy
      • Paraspinous muscle amyotrophy
      • Cervical amyotrophy
Motor Neuron Disease
   Paraneoplastic motor neuro(no)pathy
      • Mild weakness: With lymphoma
      • Severe weakness: With breast cancer
   Hopkins' syndrome: Acute post-asthmatic
   Polio & Post-polio syndrome
   SMN2 (SMNC) deletions
   Neurofibromatosis, Type 25
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
Motor Neuron Disease

 Rapid onset
  Acute Axonal Motor Neuropathy
 (with Campylobacter jejuni or serum IgG vs
      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
Motor Neuron Disease

Motor Neuron Disease

 Toxic: Lead; Dapsone; Botulism; Tick Paralysis
   West Nile
   Central European encephalitis
      • Amyotrophy
      • Polyneuropathy (± Demyelinating)
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.
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-
          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
Seasonal distribution of Guillain-Barré syndrome

  Lyu, R.-K. et al. J Neurol Neurosurg Psychiatry 1997;63:494-500
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.
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
     Rantala H, Uhari M, Niemela M. Arch Dis Child. 1991 Jun;66(6):706-8; discussion 708-9.
Subacute Inflammatory Demyelinating

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.
Chronic Inflammatory Demyelinating

A low frequency of antecedent events.
Weakness accompanied by sensory loss
 and diminished tendon reflexes
Pain and cranial neuropathies are
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
Causes Inflammatory
GB Syndrome            58%
Chronic inflammatory demyelinating
 polyneuropathy         31%
Associated with collagen
 vascular disease       7%
Other immune/infectous
  disorders             4%
asthmatic amyotrophy
 After acute asthmatic attack: Latency 1 to 18
 Mild pain: Limb, neck or meningismus
 Rapid onset weakness
asthmatic amyotrophy

  Single limb; Asymmetric; May be Proximal >
  Severity: Mild to severe
  Arm or leg
Sensory: Normal
asthmatic amyotrophy

  Protein: ± Increased
MRI: May show signal (T2) in spinal cord
Prognosis: Permanent paralysis
Flaccid or hyper tonicity

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