LMNL /floppy infant

Task : examine lower limb of the baby:

1. G. look :
- observation : head lag / Frog like Posture / Alert / Bell shaped chest./see –
saw respiration.
- Equipments : O2, wheel chairs.
- Growth
- Dysmorphism

2. inspect for 7 things :

- Muscle bulk : calf hypertrophy ….DMD.
- Posture : frog like
- Movement : fasculation.
- Contracture
- Scar .
- Leg descripency.

3. gait - if pt can walk :

- waddling gait indicate : DMD , SMA
- high steppage gait : HMSN.
- if pt. cannot walk (infant / spina bifida / SMA.)….do TPR.

4. T.P.R.( L.M.N. lesion)

- Tone: hypotonia
- Power : reduced.
- Reflexes : absent

5. then look for back and tongue , calf hypertrophy , grip hand of pt :
- Look to the Back :
 If ugly scar ……this spina bifida , then examine head for hydrocephalus.
 If no scar in the back …Look to tongue .
- Look to the tongue
 If fasculation this … SMA.
 If no fasculation ….. look to calf
- Look to the calf :
 If calf hypertrophy this ……….DMD.
 If no calf hypertrophy …shake hand …if fail to relax means …..myotonic
dystrophy

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 6. To complete my exam I would like to examine:
- CRANIAL NERVES : esp. 7th CN (in GBS)
- Check for sensation.

discussion

∆∆ of floppy infant :- ( central and peripheral causes)

A. Central : only floppy except CP ( floppy +weak)

1. Brain :
 encephalopathy , ICH .
 degenerative disease : infantile gaucher , zellweger's.
2. Spinal cord:
 spina bifida
 transaction of spinal cord , haematoma , tumor.

B. Peripheral : floppy and weak.

3. Anterior horn cell :
 Werdnig-Hoffman D - AR(Alert / Frog like position /Bell shaped chest).
 Poliomyelitis .
4. Nerve fibre :
 GBS.
5. Neuromuscular junction :
 transient neonatal myasthenia gravis
 botulism
6. muscle :
 congenital Myotonic Dystrophy - AD (mother – expressionless face/ ↑
Grip/avoid G. Anesthesia )
 Cong- Myopathy
 Congenital muscular dystrophy.
 Pompes disease.
7. Others :
 Prader-willi Syndrome.
 Down Synd.
 Chronic illness : hypercalcaemia , RTA , rickets , hypothyroidism , coelic
disease , cystic fibrosis , failure to thrive.

∆∆ OF LMNL in older child (neuromuscular disease )

1. Anterior horn cell :

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  SMA II,III (tongue fasculation)
 Polyomyilitis ( asymmetrical weakness)
2. Nerve fibre :
 HMSN or peroneal muscular atrophy.
 GBS
 Leukodystrophy .
 Posions.

3. Neuromuscular junction : myasthenia gravis.

4. Muscle :
 Muscular dystrophy (Duchene , becker's , fascioscapulohumral , limb
girdle dystrophy)
 Myotonic Dystrophy - AD (mother – expressionless face/ ↑ Grip/avoid
G. Anesthesia )
 Cong- Myopathy
 Inflammatory : dermatomyositis
 Metabolic : GSD.
 Thyroid
 Steroids.

Investigation of flopy infant :

1. chemistry :

 S. Ca ……HYPERCALCIAMIA/RICKET.
 U&E ……..RTA
 T.F.T. ………HYPOTHYRODISM.

2. Metabolic screen….ZELLWEGERS/GAUCHER .

3. TORCH Screen.

4. Chromosomal Analyses. (Prader W.S. / Down S. )

5. others :

Dd If Only Lower Limb Affected :

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 - Transverse myilitis (sensory level here).
- Spina bifida .
- HMSN
- Early GBS
- Poliomyelitis.

Dd if upper limb only affected :

- Brachial plexuses injury "erb's palsy , klump's" ……give unilateral LMNL.

- Syringomylia

Management : MDT according to cause.:

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