Dr. Athal Humo 2016
Floppy infant refers to those children presenting with generalized hypotonia, most often arising out of an insult occurred during fetal or neonatal period.Presenting Feature
Hypotonia Abnormal posture Diminished resistance to passive movement & abnormal range of joint movement Delay in motor milestonePosture
The floppy infant assumes a frog legged position. On ventral suspension, the baby can not maintain limb posture against gravity and assumes the position of a rag doll.Movements
The muscles appear flabby. There is diminished resistance to passive movement of the limbs and the range of movement of the peripheral joints is increased.Scarf Sign:Put the child in a supine position and hold one of the infant’s hands. Try to put it around the neck as far as possible around the opposite shoulder. Observe how far the elbow goes across the body. In a floppy infant, the elbow easily crosses the midline.
Pull to sit When pulled up from the supine to the sitting position, the head of the baby lags.
Delay in motor milestone
Hx. : Prenatal: infection,movement of fetus in utero, drug intake by mother during pregnancy or labour e.g. Mg sulfate, benzodiazepines, anasthesia.Perinatal: birth injury, asphyxia.Postnatal: prematurity, fit, jaundice, CNS infection .Examination :General: dymorphic features(Down), HSM, observe the strength of crying, eye for cataract.CNS:Awareness of the baby: bright eyes, follow object, smile cortical intact.Light response, response to bell & tactile.Gag & corneal reflex.Position of the limbs.Reflexes. Approach to Neonatal Immobility:
If the clinical exam. doesn't settle the question then EEG will be helpful
EEGSever slowing of EEG
Normal EEG
Cerebral disease
Spinal cord dis. or neuromuscular dis.
Floppy Baby
FB + major weaknessFB without major weakness
Awareness intact
Awareness not intact
Floppy baby with weakness:Awareness not intact (central depression):Severe brain illness: as infection,metabolic…Intoxication through mother: as barbiturate, general anasthesia…Metabolic abnormalities: as hypoglycemia, kernicterus…Characterized by:Baby not alert.Normal or increase DTR.Weak cry.+/- seizure.Awareness Intact:Spinal cord lesion: as tumor, trauma, infection, malformation ...Anterior horn cell disease: as SMA, polio …Peripheral neuropathy: as GBS, toxins as lead, drugs…Neuromuscular junction: as mysthenia gravis, botulism, aminoglycoside.Muscle disease: as congenital muscle dystrophy, GSD…
Floppy baby with no weakness: Characterized by: They appear to move well if they are supine but floppy when handled in prone position. They will be like inverted U- shape. Delay mile stones. Acute systemic illness. Mental retardation: as Down syndrome( a) Prader-willi syndrome(b) Connective tissue disorder: Ehlers-Danlos syndrome Marfan syndrome
Look for Sepsis
Nutritional-metabolic disease: Rickets Celiac disease Benign congenital hypotonia: Exhibit the condition at 6-12 months. Delayed gross motor milestone. Normal social , intelligence , fine motor movement. Head lag , slip-through ventral suspension. Complete lab. investigation are necessary, it is often unrevealing. Most of children become normal by 3 yrs. F.Hx. often +ve.
Investigation
SERUM ENZYMES: The CK level is characteristically elevated in certain diseases, such as Duchenne muscular dystrophy, and the magnitude of increase is characteristic for particular diseases. MOLECULAR GENETIC MARKERS: Many DNA markers of hereditary myopathies and neuropathies are available from blood samples. NERVE CONDUCTION VELOCITY (NCV). ELECTROMYOGRAPHY (EMG): Characteristic EMG patterns distinguish denervation from myopathic involvement. IMAGING OF MUSCLE: Imaging of muscle using ultrasonography, CT scans, and MRI are used in many neuromuscular diseases. MUSCLE BIOPSY: The muscle biopsy is the most important and specific diagnostic study of most neuromuscular disorders. NERVE BIOPSY. TEST OF IEM (serum & urine chromatography) PHYSOSTIGMINE TEST. ELECTROCARDIOGRAPHY (ECG): Cardiac evaluation is important if myopathy is suspected because of involvement of the heart in muscular dystrophies and in inflammatory and metabolic myopathies.Ataxia is the inability to make smooth, accurate, and coordinated movements,It is either acute or chronic. Usual symptoms are broad-based unsteady gait & tremor , that worsen by intentional movement of limbs (dysmetria). Defect in: Cerebellum and cerebellar pathways. PN lesion causing loss of proprioception. Bilateral frontal lobe lesions