Neonatal hypotonia, or low muscle tone, can be central or peripheral in origin. Central hypotonia presents as a "floppy but strong" newborn with normal or hyperreflexia, while peripheral hypotonia appears "floppy but weak" with possible hyporeflexia. The document outlines the clinical features, etiologies, investigations, and primitive reflexes seen in neonatal hypotonia to guide physicians in determining the underlying cause.
Neonatal hypotonia, or low muscle tone, can be central or peripheral in origin. Central hypotonia presents as a "floppy but strong" newborn with normal or hyperreflexia, while peripheral hypotonia appears "floppy but weak" with possible hyporeflexia. The document outlines the clinical features, etiologies, investigations, and primitive reflexes seen in neonatal hypotonia to guide physicians in determining the underlying cause.
Neonatal hypotonia, or low muscle tone, can be central or peripheral in origin. Central hypotonia presents as a "floppy but strong" newborn with normal or hyperreflexia, while peripheral hypotonia appears "floppy but weak" with possible hyporeflexia. The document outlines the clinical features, etiologies, investigations, and primitive reflexes seen in neonatal hypotonia to guide physicians in determining the underlying cause.
Neonatal hypotonia, or low muscle tone, can be central or peripheral in origin. Central hypotonia presents as a "floppy but strong" newborn with normal or hyperreflexia, while peripheral hypotonia appears "floppy but weak" with possible hyporeflexia. The document outlines the clinical features, etiologies, investigations, and primitive reflexes seen in neonatal hypotonia to guide physicians in determining the underlying cause.
Neonatal hypotonia is defined as CLINICAL PRESENTATION
poor tone in the muscles of the CENTRAL HYPOTONIA PERIPHERAL HYPOTONIA trunk, limbs and/or face. This “Floppy but strong” “Floppy but weak” means that the muscles provide little resistance when passively q Hypotonic posture but may q Frog-leg posture moved. Hypotonia can be respond to external stimuli q Reflexes may be normal or categorized as central or peripheral (see clinical presentation). It can with appropriate power hyporeflexive also be categorized as axial or q Reflexes normal or hyper- q Diffusely low muscle bulk truncal, predominantly affecting the reflexive and/or congenital contractures neck and spinal muscles; q Alertness and consciousness q Often show other CNS appendicular, affecting are preserved predominantly the extremities; or abnormalities: decreased level q Symmetrical or asymmetrical global. It may be identified early in of consciousness, seizures, pattern of weakness life when the newborn is unable to apnea, feeding difficulties, and obtain a normal posture during head shape abnormalities movement or at rest. q +/- Dysmorphic features
HISTORY DIFFERENTIAL DIAGNOSIS
q Timing and progression of hypotonia: acute vs chronic Acute Chronic q Prenatal history: abnormalities on US (polyhydramnios), chromosomal abnormalities, drug Systemic illness Genetic conditions exposures, infections during pregnancy, gestational q Sepsis/infection (ie. q Prader-Willi syndrome meningitis) q Down syndrome diabetes, hypertension, fetal movements. q Birth history: gestational age, mode of delivery, Metabolic conditions Neurological disorders forceps/vacuum, resuscitation, previous p9regnancies q Hypokalemia q Central q Systemic illness: GBS status, prolonged rupture of q Hypophosphatemia q Hypoxic-ischemic membranes, maternal fever, electrolyte abnormalities q Hypocalcemia encephalopathy q Genetic causes: family history, especially neurological q Hypo/hypernatremia q Malformations of brain development Genetic conditions q Intracranial bleeds PHYSICAL EXAM q Prader-Willi syndrome or strokes q Down syndrome q General Appearance: Vital signs, level of q Peripheral q Spinal muscular consciousness, signs of systemic illness, contractures atrophy q Skin: rashes, jaundice, cyanosis q Myasthenia gravis q HEENT: dysmorphic features q Congenital q Neuro: fasciculations, CN exam, primitive and distal myopathies or reflexes, observe spontaneous movements muscular q Horizontal and vertical suspension, traction response dystrophies
PRIMITIVE REFLEXES* INVESTIGATIONS
q Palmar grasp (a) q Galant (f) Guided by history and physical. q Plantar grasp (b) q Landau (g) Systemic illness: septic workup, lytes, LFTs, ammonia, lactate q Rooting (c) q Parachute (h) Neurologic causes: MRI brain +/- EEG; CK, q Moro (d) q Positive support (i) electromyography/nerve condition study, muscle biopsy. q Asymmetric tonic q Placing and Genetic/metabolic: karyotype and microarray analysis, genetic neck (e) stepping (j) testing for specific disorders, newborn metabolic screen and further *See note on Primitive Reflexes for more information. metabolic testing as indicated.
(a) (b) (c) (d) (e) (f) (g) (h) (j)
Published March, 2021
Morgan Gregg (Medical Student, University of Alberta) and Dr. Nicole Anderson (Neonatal-Perinatal Fellow, University of Alberta) for www.pedscases.com.