Pediatric Neurology Quick Talks Hypotonia Michael Babcock Summer

Pediatric Neurology Quick Talks Hypotonia Michael Babcock Summer

Pediatric Neurology Quick Talks Hypotonia Michael Babcock Summer 2013 Scenario 2 do M in the NICU Poor feeding and weakness

Not intubated Delivered 37 weeks by C/S failure to progress Poor maternal pre-natal care HC ~50% Exam axillary slippage, reduced spontaneous movements, +head lag, normal suck. +awake Hypotonia Localize > Central or Peripheral Central (brain/spinal cord) Normal/mild weakness normal bulk normal/increased reflexes Dysmorphisms encephalopathy

Peripheral (Anterior horn, peripheral nerve, NMJ, muscle) Marked weakness decreased bulk decreased reflexes no dysmorphisms Awake, alert Central Causes

Sepsis Maternal narcotics Hypothyroid Prematurity HIE Down's Syndrome Prader-Willi Inborn Errors of Metabolism Zellweger Cerebral dysgenesis Peripheral Causes

Anterior Horn SMA Pompe Peripheral Nerve (uncommon) Inflammatory GBS Demyelinating Axonal Metabolic- Leigh Neuromuscular Junction

Myasthenias Infantile botulism Hypermagnesemia Muscle Myopathy Muscular dystrophy Myotonic dystrophy Metabolic myopathy Perkowski's top 5 not to miss causes of floppy baby

Down's syndrome Prader Willi Pompe (have heart problems) Zellweger Spinal Muscular Atrophy However, most common is HIE History Mother systemic illness, fever, substance abuse Pregnancy polyhydramnios, fetal movement, abnormal lie Delivery complicated/prolonged, trauma, Apgars Family history delayed milestones, weakness, myotonia

Exam Assess Tone Tone is resistance to stretch forces, develops with nervous system development, low tone is normal for premature infants Ballard testing. Resting posture assess resting posture when infant is quiet/drowsy Hypotonic infant frog leg position Long-standing immobility can cause joint contractures arthrogryposis Passive manipulation Infants develop increasing flexor tone in extremities causes increased recoil after limb is extended Head control in vertical/horizontal suspension

Vertical suspension Ballard Testing Testing tone Work Up Central Electrolytes (Mg, Ca, Gluc) TFT's Brain imaging U/S vs MRI EEG Karyotype, CMA Metabolic work-up

Peripheral CK EMG/NCS muscle/nerve bx Spinal Muscular Atrophy (SMA) AR SMN1 gene SMN protein

(survival motor neuron); SMN2 gene regulates severity Weakness and atrophy of muscles, including tongue Symmetric weakness, more proximal than distal, more severe in LE Tongue fasciculations Absent DTRs Normal intellectual capacity Facial muscles typically spared early on Types Type 1 infantile - Werdnig

Hoffman - <6mo. - never sit Type 2 intermediate Dubowitz 6-18 months never walk Type 3 juvenile Kugelberg-Welander 18mo- 17 years. - able to walk initially, often lose this. Type 4 adult-onset Myasthenias Congenital Genetic disorder of NMJ Hypotonia, weakness (ocular, bulbar, respiratory) Variable onset, sometimes in utero arthrogryposis

Recurrent ALTEs Fatigability, weak cry, feeding difficulties, episodic apnea Sometimes respond to AchE inhibitors Transient neonatal Transplacental transfer of AchR Antibodies. 10-15% of infant of myasthenic mothers Hypotonia, weaknessbulbar and respiratory, within 4 days of birth Good response to AchE inhibitors Infant Botulism

Weakness and hypotonia Can have hx of honey ingestion; though contamination from soil is most common Constipation is often first sign Eye findings, ophthalmoplegia botulism is descending paralysis Can test stool Can give Human boutlinum immunoglobulin 50mg/kg in first few days shortens course Often severe respiratory weakness requiring ventilation, prolonged course. Prep Question During the health supervision visit for a 6 week old boy, his father expresses concern that his son doesnt look like his other children. Growth parameters are normal except for a head circumference of 35.5 cm (<5th percentile). On PE, you note that the infant does not appear to fixate or track your face visually. There is a slip through on vertical suspension and draping over on horizontal

suspension. DTRs are brisk. Moro reflex is present and brisk. Of the following, the MOST likely cause of this infants hypotonia is: Anterior horn cell disease Congenital brain malformation Congenital myasthenic syndrome Congenital myopathy Spinal cord disease B. Congenital brain malformation

Hypotonia Localize! UMN vs. LMN signs, axial vs appendicular Take into account growth parameters, especially HC, as well as features such as tracking Regarding other choices: A. anterior horn cell disease wouldn't cause microcephaly or increased reflexes C. Congenital myasthenic syndrome wouldn't cause microcephaly or brisk reflexes D. Congenital myopathy no microcephaly or poor visual tracking E. Spinal cord disease wouldn't cause microcephaly or poor visual tracking. References

Paediatr Child Health. 2005 September; 10(7): 397400.; PMCID: PMC2722561; A schematic approach to hypotonia in infancy Respiratory update.com

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