Paramount to determining
the anatomical diagnosis is the distinction between upper motor neuron
system and motor-sensory unit dysfunction. This distinction can often
be made based on the evaluation of the dynamic tone. Dynamic tone refers
to the response of the striated muscles to being stretched by a brief
high-intensity force. The dynamic tone is evaluated
by determining: (1) the resistance the limbs offer to the examiner’s
effort to quickly extend them, and their speed of recoil after such
a maneuver; (2) the characteristics of the Moro and stretch muscle reflexes;
and (3) the presence of clonus.
The limbs of neonates with increased dynamic tone offer increased resistance
to the examiner’s effort to quickly extend them, and have a quick recoil
once the limb is released. Moro reflex is exaggerated, muscle stretch
reflexes are increased, radiating stretch reflexes (cross adductor response)
are evident, and clonus may be present in the limbs and jaw of neonates
with hypotonia and increased dynamic tone. Neonates with hypotonia and
increased dynamic tone do not give the appearance of weakness because
of the forceful displacement of the limbs during these maneuvers. Neonates
with hypotonia and increased dynamic tone often have cortical thumbs.
The site of pathology in neonates
with hypotonia and increased dynamic tone is the upper motor neuron:
either in the brain, brainstem, rostral spinal cord, or a combination
of these sites. The distinction among brain, brainstem, or spinal cord
hypotonia can often be made based on associated
neurological findings such as seizures, weakness of facial muscles,
increased facial dynamic tone, parasympathetic pupil abnormalities,
lack of bowel movements, and anal sphincter weakness (Figure 98.1).
Figure 98.1.—
Schematic
representation of the possible sites of neuromuscular damage in neonates
with generalized hypotonia and increased dynamic tone. The symbols in
the rectangular box depict the presence (in green background) and the
absence (in red background) of important neurological findings. These
findings help to localize the site of damage in hypotonic neonates:
(1) brain; (2) brainstem; and (3) rostral spinal cord.
The limbs of neonates with decreased dynamic tone offer little resistance
to the examiner’s effort to quickly extend them and they have poor recoil.
Moro and muscle stretch reflexes are diminished in neonates with hypotonia
and decreased dynamic tone. Clonus is not present. Neonates with decreased
dynamic tone are weak. Cortical thumbs are not present.