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are more than those that affect the lower neuron, so the most
common cause of neonatal Hypotonia is brain problems and those patient with
brain problems after 6-9 months of age we'll start see spasticity in them,
this means that after 6 months he'll start to have fisting and scissoring and
in the same time he will have head lag which means a combination of central
Hypotonia and limp spasticity and this is a typical characteristic of insult to a
developing brain. Most insults are prenatal or within the first year after life.
Now, the most important thing to do when you find a baby with Hypotonia is
to differentiate if it is upper or lower motor neuron problem and the most
common is a brain problem which is affected by acquired diseases more than
motor units.
So what are the clues to differentiate between them?
Cerebral Hypotonia: in addition to having Hypotonia the patient also has:
1. Abnormalities of other brain functions: like he cant concentrate on
your face or having seizers.
2. Dysmorphic features: like micro or macro cephaly.
3. Fisting of the hands: while in motor unit problems there is weakness in
fisting.
4. Malformations of other organs: like cleft lip or cleft palate.
5. Movement through postural reflexes.
6. Normal or brisk tendon reflexes.
7. Scissoring on vertical suspension with spasticity.
So the most important thing in cerebral Hypotonia is there is evidence of
cerebral dysfunction while in motor unite Hypotonia the baby is alert, he only
has motor delay.
So motor unit Hypotonia is characterized by:
1. Normal brain functions: this is the most important one.
2. No abnormalities of other organs.
3. Absent or depressed tendon reflexes.
4. Failure of movement on postural reflexes.
5. Fasciculations.
6. Muscle atrophy.
And here in lower motor neuron problems the baby is hypotonic and weak
while in upper the baby is hypotonic but slightly weak which means when you
start playing with him he starts moving his limbs.
So, how to assess the weakness in infants? By looking at the movement of
the child (antigravity movement).
DD-X to cerebral Hypotonia: anything that affects the brain will result in
cerebral Hypotonia like:
1. CNS infections: TORCH, acquired CNS infections.
2. Chromosomal abnormalities, like:
- Down syndrome.
- Prader - Willi syndrome: hypotonic and Dysmorphic.
3. Cerebral malformations: like holoprosencephaly or Lissencephaly
(smooth brain) and others.
4. Hypoxic ischemic encephalopathy: either pre or post natal.
5. Inborn errors of intermediary metabolism, like:
- Peroxisomal disorders (Zellweger Syndrome).
- Acid maltase deficiency (Pompes disease).
Usually, we cant treat a brain insult after it occurs and we have to prevent
it. The outcome of Hypotonia in the future depends on the severity of the
insult to the brain and the underlying cause of Hypotonia; which means if it is
not severe then the patient will have a delay in acquiring milestones, on the
other hand, if it is severe the patient will not acquire the ability to sit or
walk. So our aim is to diagnose diseases early to prevent brain insult.
As we know, the neuraxis starts from the brain through the spinal cord and
nerves till muscles, any problem in this pathway in infant will give you
Hypotonia.
An examples of it in the brain is Prader-Willi Syndrome, in the spinal cord is
hypoxic ischemic myelopathy, hyper twisting injury of the spinal cord during
delivery, problems in the peripheral nerves in perinatal period is uncommon,
congenital myasthenic syndrome in the neuromuscular junction, congenital
muscular dystrophies, all of these will give you neonatal Hypotonia.
Clinical picture:
1) Lower motor neuron lesion:
a. Hypotonia and flaccid weakness: they are the weakest and the most
floppy infant you will see, they dont have any antigravity movement.
b. Decreased or absent deep tendon reflexes
c. Fasciculations: always a sign of anterior horn cells disease either in
children or adults so we see them also in polio, so always fasciculation
and weakness mean anterior horn problem. In SMA, it is mostly seen
in the tongue and look like a bag of worms.
d. Atrophy.
All the muscles in the body are affected except eye muscles, so you see
these patients usually intubated, alert, awake and cant move their muscles
except eye muscles.
The previous analysis is applied to type 1 SMA.
SMA type 2 is autosomal recessive, onset is after 6 months and less than
18 months and the life span is longer because they dont develop severe
respiratory failure.
SMA type 3 is the best, they have normal life span because they dont
develop respiratory muscles failure.
evidence
of
denervation
NCS/EMG
(nerve
conduction
study/
electromyography).
acquired
neonatal
myasthenia:
when
the
mother
has
The end