Floppy Infant

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Floppy Infant

• Prof. dr. Shahenaz M. Hussein

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Brain
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Spinal cord
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• Hypotonia
• Clinical features
• In early infancy, there is decreased muscular
  activities both spontaneous and in response to
  postural reflex testing and to passive motion.
• In young infants , hypotonia manifested as Frog
  posture.
• In older infants, there is delay in motor
  milestones.
• Ventral suspension In this position floppy
  infant droops over the hand like an inverted U
  shape.
• Head lag when pulled up from supine to sitting
  position.

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• Clinical features
• Weak cry
• Abdominal breathing.
• No or depressed tendon reflexes.
• Fasciculation's of the tongue.
• Normal mentality in infantile progressive
  muscular atrophy
• Mental retardation in congenital muscular
  dystrophy.

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Lower Motor Neuron
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• Etiology
• A- Paralytic causes Lesion of the lower motor
  neuron.
• 1- Infantile progressive muscular atrophy
  (IPSMA) Decreased movement in-utero Gradual
  weakness Delay in gross motor milestones- Weak
  cry Absent deep tendon reflexes
  Fasciculation's of tongue- Normal personal and
  social behaviors.
• 2- Infantile botulism Poor feeding,
  constipation, failure to thrive, facial weakness,
  ptosis- ocular palsy, inability to suck or
  swallow, apnea (Source soil dust, honey).

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• Etiology
• 3-Myasthenia gravis (Neonatal transient and
  congenital persistent) Poor suckling- chocking-
  respiratory distress- weak cry-mother may have
  myasthenia.
• 4- Myotonic dystrophy AD, Polyhydramnios,
  failure of suckling, facial diplegia,
  arthrogryposis, thin ribs, developmental delay.

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Myathenia gravis
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• Etiology continue
• 5- Congenital muscular dystrophy Early onset
  facial weakness, joint contractures, mental
  retardation, seizures.
• 6- Rare types of myopathy (Nemaline and Central
  core) Proximal weakness( shoulders, hips),but
  the finger movement is preserved, intelligence,
  fine motor, language and social milestones are
  normal.
• 7- Spinal cord lesion Upper thoracic spinal cord
  injury after breech extraction.
• 8- Glycogenosis Type II glycogen storage pompe
  disease, type V McArdle disease).

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• B- Non-Paralytic causes
• 1- CNS disorders Atonic diplegia,
  choeoathetosis , ataxic and atonic cerebral
  palsy.
• 2- Syndromes with hypotonia
• 1-Tisomy 21.
• 2- Prader willi syndrome (deletion of
  15q11 gene)
• 3- Marfan syndrome.
• 4- Turner syndrome.
• 3- Degenerative disorders
• -Tay-Sacks disease.
• - Metachromatic leukodystrophy.

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• Non- paralytic causes Continue
• 4- Systemic diseases
• -Malnutrition.
• -Celiac disease, and Cystic.Fibrosis.
• - Chronic illness .
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  Metabolic diseases Hypercalcemia.
• - Endocrinopathy Hypothyroidism.
• 5- Benign congenital hypotonia
• mild to moderate hypotonia with weakness.

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Celiac Disease
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Hypothyroidism
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• Differential diagnosis of flaccid paralysis
  (LMNL)
• 1-Guillain Barre syndrome (postinfectious
  polyneuritis) It is the most common cause of
  acute paralysis in children/Although infants can
  be affected, most cases are seen in children
  above the age of 3 years. History of preceding
  viral infection, one or two weeks before the
  onset of ascending symmetrical paralysis, is
  usually obtained.
• 2-Poliomyelitis It is going to be eradicated in
  many countries of the world. The spinal lesion
  lead to Asymmetrical flaccid paralysis.

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• Differential diagnosis of flaccid paralysis
  (LMNL)
• 3-Post diphtheritic paralysis This form of toxic
  polyneuritis is uncommon nowadays because of the
  routine vaccination.
• History of preceding sore throat and
  swollen neck (pharyngeal diphtheria) or stridor
  (laryngeal diphtheria), 1 - 2 week before the
  onset of paralysis can be obtained.
• 4-Transverse myelitis It is the second most
  common cause of acute symmetric paralysis.
• -It is a segmental dysfunction of the spinal
  cord without an evidence of spinal cord
  compression. -The cause is probably either a
  direct viral infection or an autoimmune disease.

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Transverse Myelitis
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• Differential diagnosis of flaccid paralysis
  (LMNL) continue
• 5- Botulism
• It is a toxic neuromuscular blockade caused by
  clostridium botulinum (anaerobic gram positive
  bacteria).
• Three forms are present
• (1) Infant botulism occurs in infants.
  Germination of spores in gastrointestinal tract
  follows exposure to soil, house dust. honey or
  corn syrup
• (2) Food-induced botulism occurs with ingestion
  of improperly home-preserved foods containing the
  toxins.
• (3) Wound botulism occurs due to wound
  contamination with the clostridium botulinum
  organisms.

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• Differential diagnosis of flaccid paralysis
  (LMNL) continue
• 6- Acute spinal cord compression Trauma to the
  back, spinal epidural abscess and vascular
  anomalies of the cord may produce acute
  paralysis.
• Occasionally, spinal cord tumors may also present
  with acute paralysis.
• Myelography is essential to demonstrate the
  obstruction. CT scan of the spine or MRI are more
  sensitive.
• 7- Hypotonia with movement disorders
• - In acute cerebellar ataxia, hypotonia is
  usually present, but the truncal ataxia with
  unsteady gait is the most characteristic feature.
• - In rheumatic chorea, hypotonia is present and
  may be marked simulating acute paralysis but
  abnormal involuntary choreic movements is the
  dominating finding.

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• Diagnosis
• Differentiate hypotonia with hyporeflexia
  from hypotonia with hyperreflexia.
• -Brisk reflexes point to cerebral dysfunction.
• -Creatine kinase level and EMG are normal in
  neurological lesions.
• -Prolonged nerve conduction in polyneuropathy.
• -Muscle biopsy to diagnose the type of myopathy.
• Management
• -Treat the cause.
• -Physiotherapy.
• -Genetic counseling.