Neurology Flashcards
What is cerebral palsy?
Central motor impairment secondary to fetal or infantile brain injury (nonprogressive but might not recognize sxs until later)
Etiologies of cerebral palsy
Hypoxia Trauma Premature birth Infections Toxins Structural abnormalities
What are the subtypes of cerebral palsy based on?
Where in the brain the problem is
Sxs of cerebral palsy
Abnormal tone/posture
Retained primitive reflexes (to 9-12 mos)
Not reaching milestones (sit by 8 mos and walk by 18)
Excessive irritability
Poor feeding/drooling
Poor visual attention
Difficult to hold, cuddle
Management of cerebral palsy
Early recognition, referral intervention
Sxs
What is the chiari type 1 malformation?
Cerebellar tonsils displaced caudally below foramen magnum (can be associated with syringomyelia which is a fluid filled cyst in the spinal cord)
Sxs of chiari malformation type 1
Might not see until teen or adult Loss of abdominal reflex HA Neurologic sxs associated with syringomyelia (cape like distribution of numbness)
What is a chiari type II malformation?
Type 1 + myelomeningocele
Usually detect prenatally or at birth
Sxs of chiari type II malformation
Hydrocephalus
Dysphagia
UE weakness
Apneic spells and aspiration
What is spina bifida occulta?
Incomplete closure of spinal canal (usually lower back)
No or mild signs
Hairy patch, dimple, dark spot, swelling on back at site of gap
What is a meningocele?
Outpouching of spinal fluid and meninges through vertebral cleft
Mild problems with sac protrusion
What is a myelomeningocele?
Most severe!
Spinal cord and nerves protrude from vertebral cleft
Weakness, loss of bladder and/or bowel control, hydrocephalus, inability to walk
Learning probs
Reasons for spinal dyraphisms
Genetics
Low folate
Meds during pregnancy
Poorly managed diabetes
Tx of spinal dyraphisms
Early recognition with US of AFP blood test
Prevention by taking folate
Neuro referral so surgery or shunt
What is hydrocephalus?
Increased volume of CSF and causing ventricular dilation and increased ICP
2 types of hydrocephalus
Obstructive (blockage)
Non-obstructive (impaired absorption or rarely overproduction)
Etiologies of hydrocephalus
CNS malformations Infection Intraventricular hemorrhage Genetic defects Trauma CNS tumors
Sxs of hydrocephalus
Asymptomatic Bradycardia, HTN, altered respiration HA, n/v, behavior Papilledema Macrocephaly Spasticity Diplopia Spinal abnormalities
Diagnostic testing for hydrocephalus
Newborns: US
Older infants/kids: MRI or CT
Management of hydrocephalus
Refer to neuro and shunt (from head to peritoneal space)
What is microcephaly?
Head circumference > 2 standard deviations below mean or <5th percentile
Primary or secondary microcephaly
Primary: lack of brain development or abnormal development due to timing of insult
Secondary: injury or insult to previously normal brain
Reasons for microcephaly
Genetic Prenatal and perinatal injury Craniosynostosis (sutures close too early) Postnatal injury Metabolic Toxin exposure
Possible sxs of microcephaly
Delayed milestones
Seizures or spasticity
Fontanelle may close early and sutures are prominent
What is macrocephaly?
Head circumference >2 SD above mean or above 95th percentile
What causes macrocephaly?
Due to increase in size of any components of cranium (brain CSF etc) Rapid growth (Increased ICP), catch up growth, normal growth rate and genetic
Manifestations of NF1
Cafe au lait macules Axillary and inguinal freckling Lisch nodules Optic glioma Neurofibromas (macrocephaly, seizures, cognitive)
Most common primary HA
Migraine and tension
Most common secondary HA
Acute febrile illness
Characteristics of a migraine
Focal, unilateral or bilateral 2-72 hrs Moderate to severe Pulsatile/throbbing Aggravated with activity or reduced activity
Characteristics of a tension HA
Diffuse, frontal or temporal (hat band distribution) 30 min-7 days Mild to moderate Constant pressure, non-throbbing Not aggravated with activity
When to worry with a HA
Abnormal neuro of visual exam Severe when wake up or awaken in middle of night Daily sxs with progressive worsening Accompanied with vomiting Acute onset with previous history Increased with coughing or bending
What is a pseudotumor cerebri?
Idiopathic intracranial HTN
Increased ICP without mass or hydrocephalus
*obese teenage girl
Sxs of pseudotumor cerebri
HA and papilledema!
Visual sxs
Visual field loss, visual acuity loss
Pulsatile tinnitus
Diagnostics for pseudotumor cerebri
Neuro eval (MRI or LP) Opthalmo
Management for pseudotumor cerebri
Manage sxs and preserve vision
Acetazolamide (reduce rate of CSF production)
Topiramate (control HA and reduce weight)
Furosemide (reduce fluid and pressure)
Weight loss, shunt fluid etc
What classifies epilepsy?
> 2 seizures occurring more than 24 hrs apart
Types of seizures
Focal/partial
Generalized
Unknown
Unclassified
Features of an absence seizure
Sudden impairment of consciousness w/o loss of tone
Provoked by hyperventilation!!
Genetic
B/w 4-10 and most spontaneous remission by puberty
Arrest in activity (9-10 sec and over 10x/day)
1st line med for absence seizure
Ethosuximide
Features of febrile seizure
Convulsion with temp >38C (100.4F)
Age 6 mos-5 yrs
Associated with virus
Maybe genetic
Simple vs complex febrile seizure
Simple: most common, <15 min
Complex: focal, >15 min or >1/24 hrs
Management for febrile seizure longer than 5 min
IV benzodiazepines
What is the most common cause of acute flaccid paralysis in healthy infant/child?
Guillian-Barre syndrome
What happens before Guillian Barre syndrome?
Illness (mostly campylobacter)
Sxs of Guillian Barre syndrome
Ascending symmetric weakness
Neuropathic pain
Gait instability or refusal to walk
Absent reflexes
Most specific diagnostic for guillian barre syndrome
Electrodiagnostic studies (EMG) Others are CSF (increased protein with normal WBC) or spinal MRI and maybe contrast
Management of guillian barre syndrome
Hospitalize
Tx with IVIG or plasma exchange
Sxs of botulism
Descending weakness
Constipation, poor feeding
Hypotonia, loss of DTRs
Irritable and lethargic
How to diagnose botulism?
Stool sample and EMG
Tx for botulism
Hospitalize
Botulism immune globulin (BIG-IV or babyBIG)
What is duchenne muscular dystrophy?
X-linked recessive disorder Defect in genes responsible for muscle function Elevated muscle enzymes Age 2-3 onset with more severe sxs Wheelchair by 13
Sxs of duchenne muscular dystrophy
Progressive weakness (proximal before distal, LE first) Gower's sign (hands to get up) Pseudohypertrophy of calves Growth delay Cognitive impairement
Associations with duchenne muscular dystrophy
Cardiomyopathy
Orthopedic complications
Cognitive impairment
Management for DMD
Glucocorticoids
Features of becker muscular dystrophy
Sxs onset later
Muscle involvement is not as severe
CK elevated over 5x more
Cardiomyopathy may be more predominant
