2025 – PAGE 396 – NEUROLOGY
HORNER SYNDROME (AKA HORNER’S)
In Horner syndrome, a T1 lesion results in ptosis, miosis, and anhidrosis. The affected eye has a droopy lid and a small pupil (anisocoria), and it is dry.
PEARL: Associated with KLUMPKE PALSY.
IMAGE: www.pbrlinks.com/HORNERS1
SPASTIC CEREBRAL PALSY (CP)
Spastic cerebral palsy (CP) is a motor impairment due to brain lesions or anomalies. The condition does NOT progress, but the motor component can change with time. The diagnosis is usually made by 1 year of age. Intelligence can be fully intact. The increased incidence is due to improved survival of preterm infants.
- SPASTIC HEMIPLEGIA: Affects upper and lower extremities on ONE side (rigid, weak, or paralyzed). Usually affects the arm more than the leg. Good cognitive prognosis.
- SPASTIC DIPLEGIA: The legs are affected, being tight and stiff. Great cognitive prognosis.
- SPASTIC QUADRIPLEGIA: All extremities are affected. This has a horrible prognosis.
- PEARL: Though asphyxia is often thought to be the most common etiology for cerebral palsy, it’s NOT. It’s actually only responsible for a small number of cases. Prematurity, IUGR, and intrauterine infections have a much higher association with cases of cerebral palsy.
ATHETOID CEREBRAL PALSY
Athetoid cerebral palsy is like spastic CP, but there is also dystonia.
MNEMONIC: Ever heard of athetoid chorea? If so, that could help you remember the odd movements related to the dystonia in athetoid cerebral palsy.
WEAKNESS AND PARALYSIS PEARL
This is extremely important! In any child who has a progressive weakness or paralysis, your first step should be to evaluate him/her for respiratory compromise. Pulse oximetry is useless. Look for tachypnea, accessory muscle use, paradoxical breathing (belly breathing), and shallow respirations to help you decide whether or not you need to choose “prepare to intubate.”
GUILLAIN-BARRE SYNDROME (GBS, AKA ACUTE INFLAMMATORY DEMYELINATING POLYNEUROPATHY or AIDP)
Patients suffering from Guillain-Barre syndrome (GBS, AKA acute inflammatory demyelinating polyneuropathy or AIDP) may initially complain of back pain and fever and can have a facial palsy and proximal muscle weakness (trouble rising from a chair or shrugging shoulders) prior to lower extremity symptoms. Classically, though, it is an ascending paralysis over several days to weeks in which there is ataxia and then an inability to walk. Look for diminished or absent reflexes in the lower extremities on exam. Sensation is preserved (as is bowel and bladder continence). It can progress to respiratory compromise requiring intubation. Perform a lumbar puncture to look for albuminocytologic dissociation (increased CSF protein in the absence of increased WBCs). FYI, they could say there is an absence of pleocytosis (pleocytosis means an increase in WBCs). For treatment, you can try IVIG or plasmapheresis. The clinical diagnosis of GBS needs to be confirmed with CSF analysis and nerve conduction studies.
PEARLS: Steroids DO NOT help. Pulse oximetry is a poor indicator of neuromuscular respiratory insufficiency. You can, however, try to obtain a negative inspiratory flow (NIF) or a Forced Vital Capacity (FVC) if the child is old enough to participate with the test (at least 5 years of age). Always keep tick paralysis in your differential, especially if they mention the summertime, a recent vacation, or the woods! Additionally, if someone presents with GBS a few weeks after a diarrheal illness, they might be referring to C. jejuni infection (a known antecedent to GBS though the mechanism is not understood). Also, when compared to any CORD COMPRESSION SYNDROME, GBS maintains rectal tone, bowel/bladder continence, and sensation. It also has decreased reflexes. In cord compression syndromes, sensation, tone, and continence are lost, and reflexes are increased.