3 Peds Neurology Flashcards
____% of kids 18 or younger experience some sort of developmental delay
18%
Common concern presenting to primary care, with many etiologies
What is important to ascertain when investigating a potential developmental delay?
You want to see a TREND over time, not a single out-of-range measurement/milestone
Non-progressive central motor impairment secondary to fetal or infantile brain injury
Cerebral Palsy
Incidence: 2 per 1,000 births
Possible etiologies of cerebral palsy
Hypoxia*** Trauma Premature birth Infections Toxins Structural abnormalities
What are the different subtypes of cerebral palsy?
SPASTIC - most common (70-80%)
Muscles appear stiff and tight, arises from MOTOR CORTEX damage
ATAXIC (6%)
Characterized by the shaky movements; affects balance and sense of positioning in space; arises from CEREBELLUM damage
DYSKINETIC (6%)
Involuntary movements, arises from BASAL GANGLIA damage
Can have a combo, sometimes not noticed until 18-24 months
SSx of Cerebral Palsy
Abnormal tone and/or posture RETAINED PRIMITIVE REFLEXES Not reaching milestones Excessive irritability Poor feeding, drooling Poor visual attention Difficult to hold, cuddle
What are some retained primitive reflexes common in cerebral palsy
ATNR (Asymmetric tonic neck reflex - the fencer’s pose) - baby turns head to extended arm; usually gone by 4-6 months
Moro Reflex - baby stretching out his arms with a startle on suddenly being released for an instant
Management of Cerebral Palsy
EARLY RECOGNITION, REFERRAL, AND INTERVENTION
Parental support and counseling
Symptomatic management
• OT, PT, bracing
• Anti-spasmodic
• Botulism toxin
Nervous system malformations are present in ____% of infants who die <1 year
40%
Type of malformation dependent on what gestational period the insult occurs
Etiologies of congenital malformations of CNS
Infections Toxins Genetic Metabolic Vascular
Cerebellum tonsils displaced causally below the forsaken magnum
Chiari Type I
Can be associated with syringomyelia (fluid filled cyst within spinal cord)
Chiari Type I
Symptoms of this CNS malformation often don’t present until teen or adult years
Chiari Type I
Loss of abdominal reflex
Neurological Sx assoc with syringomyelia
Cape-like numbness
Chiari Type I
What’s the other name for Chiari Type II?
Arnold-Chiari malformation
It’s a Type I + myelomeningocele
CNS malformation usually detected prenatally or at birth
Chiari Type II
Hydrocephalus
Dysphagia
UE weakness
Apneic spells and aspiration
Chiari Type II
What are the three types of Spinal Dysraphism?
Spina Bifida Occulta (most mild)
Meningocele
Myelomeningocele (most severe)
Incomplete closure of the spinal canal, usually on the lower back but can be anywhere
Spina Bifida Occulta
Hairy patch, lumbar dimple, dark spot, swelling on the back at the site of the gap in the spine
Spina Bifida Occulta
Outpouching of the spinal fluid and meninges through a vertebral cleft
Meningocele
Mild problems associated with the sac protrusion
Spinal cord and/or nerves protrude from the vertebral cleft
Myelomeningocele
Most severe form of spina bifida
Weakness, loss of bladder and/or bowel control, hydrocephalus, and inability to walk result
Spinal Dysraphisms can occur anywhere along the spine but _________ is most common
Lumbar spine
What are some common etiologies of spina bifida
Genetics
LOW FOLATE***
Medications during pregnancy (esp anti seizure meds)
Poorly managed diabetes
Early recognition of spina bifida is possible by…
U/S
Alpha-feto protein markers
Best way to prevent Spina Bifida
Prenatal vitamins including folic acid 3 months prior to conception
Increased volume of CSF causing ventricular dilation and increased intracranial pressure
Hydrocephalus
What are the two types of hydrocephalus?
Obstructive - due to blockage
Non-obstructive - impaired absorption or (rarely) overproduction
Etiologies of Hydrocephalus
CNS malformations Infection Intraventricular hemorrhage Genetic defects Trauma CNS tumors
SSx of hydrocephalus
(Can be asymptomatic) Bradycardia, HTN, altered respiratory rate HA, N/V, behavior changes Papilledema Macrocephaly Spasticity Diplopia Spinal abnormalities
How is hydrocephalus diagnosed?
Newborns/infants: U/S
Older infants/children - MRI or CT
Treatment for hydrocephalus
Refer to neurosurgeon
Shunt
What is the definition of Microcephaly
Head circumference ≥2 standard deviations below average or <5th percentile
Can be primary (congenital) or secondary (postnatal)
Lack of brain development or abnormal development due to timing of insult
Primary microcephaly
Injury or insult to a previously normal brain
Secondary (postnatal) microcephaly
If microcephaly is symptomatic, what might you see?
Delayed milestones
Seizures or spasticity
Fontanelle may close early and sutures may be prominent
What is the definition of Macrocephaly?
Head circumference ≥2 standard deviation above average or >95th percentile
Can be due to increase in size of any components of cranium (brain, CSF, blood, or bone)
Rapid growth —> macrocephaly is suggestive of…
Increased intracranial pressure
Catch-up growth macrocephaly is typically seen in…
Premature infants (neurologically intact)
Normal growth rate macrocephaly is usually the result of …
Familial macrocephaly or megaloencephaly
How are micro- and macrocephaly managed?
Neurology referral
Labs/imaging
Treat underlying cause
Inheritance pattern for Neurofibromatosis (NF1)
Autosomal dominant
What neurological abnormalities are associated with neurofibromatosis?
Macrocephaly, seizures, cognitive defects
Clinical manifestations of NF1
Cafe-au-lait macules (appear 1st year)
Axillary and/or inguinal freckling (3-5 years)
Lisch nodules
Optic gloom a
Neurofibromas
Most common types of primary headache
Migraine and tension
Most common secondary headache
Acute Febrile Illness
Migraine or Tension H/A:
Focal, unilateral or bilateral
Migraine
Migraine or Tension H/A:
Diffuse, frontal or temporal
Tension
Migraine or Tension H/A:
Duration = 2-72 hours
Migraine
Migraine or Tension H/A:
Duration = 30 min - 7 days
Tension
Migraine or Tension H/A:
Moderate to severe intensity, pulsatile/throbbing
Migraine
Migraine or Tension H/A:
Mild to moderate intensity, constant pressure/non-throbbing
Tension
Migraine or Tension H/A:
Aggravated with activity or reduced activity
Migraine
Migraine or Tension H/A:
Not aggravated with activity
Tension
When to worry about a headache
Abnormal neurologic or visual exam
Severe upon awakening or awaken in the middle of the night
Daily symptoms with progressive worsening
Accompanied with vomiting
Acute onset with previous history
Increased with coughing or bending
Idiopathic intracranial hypertension without mass or hydrocephalus, typically seen in obese teenage girls
Pseudotumor cerebri
SSx of Pseudotumor Cerebri
HA*** Papilledema*** Visual symptoms Visual field loss, visual acuity loss Pulsatile tinnitus
How is pseudotumor cerebri diagnosed?
Neuro eval —> MRI to exclude secondary causes, LP
Ophthalmologic eval
What will an LP show in pseudotumor cerebri?
Elevated opening pressure
Treatment of pseudotumor cerebri is focused on …
Managing symptoms and preserving vision
DOC - ACETAZOLAMIDE to reduce rate of CSF production
Topiramate to help control HA and reduce weight
Furosemide to reduce fluid and pressure
Weight loss
Shunting of fluid
Optic nerve fenestrations
Sudden, transient disturbance of brain function manifested by involuntary sensory, motor, autonomic symptoms with or without loss of consciousness
Seizure
What is the definition of epilepsy?
≥ 2 seizures occurring more than 24 hours apart
Types: Focal (partial) - with or without impaired awareness Generalized Unknown Unclassified
Sudden impairment of consciousness w/o loss of tone, provoked by hyperventilation and presumed to be genetic
Absence Seizures
Presents between age 4-10 and most often spontaneously remits by puberty
Arrest in activity generally lasts 9-10 sec and may occur 10x/day
1st line medication for absence seizures
Ethosuximide
Convulsion w/ temp > 38˚C (100.4˚F) in a child age 6 months to 5 years
Febrile seizure
Often associated with viral infection, genetic predisposition
Most common type of febrile seizure, lasting <15 min
Simple febrile seizure
How are complex febrile seizures defined?
Focal, last >15 min or occur >1/24 hours
How do you manage a febrile seizure
Generally self-limiting
If >5 min, give IV benzodiazepines
Acute immune-mediated polyneuropathy preceded by illness (most commonly campylobacter)
Guillian-Barre Syndrome
Most common cause of acute flaccid paralysis in health infants/children
Guillian-Barre Syndrome
ASCENDING symmetric weakness, neuropathic pain, gait instability/refusal to walk, and absent reflexes
Guillian-Barre Syndrome
What is the primary diagnostic tool for Guillian-Barre syndrome?
Electrodiagnostic studies (EMG)
Can also do CSF analysis or Spinal MRI
Treatment of Guillian-Barre Syndrome
Hospitalization and close monitoring
Treatment with IVIG or plasma exchange
Honey and home canning are no-nos because of …
Risk of ingesting clostridium botulinum spores —> botulism
90% of infants with botulism are _______
<6 months old
DESCENDING weakness
Constipation, poor feeding
Hypotonia, loss of DTRs
Irritable and lethargic
Botulism
How is botulism diagnosed?
Stool sample
EMG
How is botulism treated?
Hospitalization and close monitoring
Botulism immune globulin (BIG-IV or BabyBIG)
Inheritance pattern for Duchenne Muscular Dystrophy
X-linked recessive
Defect is in genes responsible for muscle function —> elevated muscle enzymes
What is the outlook for kids with DMD?
Wheelchair bound by age 13 and mortality by 18-20 years
Progressive weakness, proximally before distally, LE before UE
Duchenne Muscular Dystrophy
What is Gower’s sign?
Using hands to push up from floor (DMD)
Conditions associated with DMD?
DCM
Orthopedic complications (ie scoliosis)
Cognitive impairment
Diagnosis of Duchenne Muscular DystrophY
Elevated muscle enzymes - CK ≥10-20x normal
Genetic testing
Treatment for DMD
GLUCOCORTICOIDS
How is Becker Muscular Dystrophy different from DMD?
Symptom onset later
Muscle involvement not as severe
CK elevated ≥5x
Cardiomyopathy may be more predominant
