Neurology Flashcards
1
Q
- teenager with frontal HA or band-like pressure sensation
- Tx: eliminating the stressor
A
Tension HA
2
Q
- HA with photophobia
- Tx: ibuprofen, APAP, fluids and rest, ergotamines, and triptans (NOT SQ)
A
Migraine HA
3
Q
- progressive and intermittent visual disturbances
- HA worse in the am, HA relieved by vomiting
- papilledema
- Dx: CT
A
Increased ICP
4
Q
- AR disease of copper metabolism
- liver cannot get rid of copper, so it accumulates in liver, kidneys, brain, and eyes
- younger pts present with liver disease
- older pts have neuro/psych Sx
- tremors, emotional problems, difficulty with handwriting, depression, and abnl eye movements
- acute hepatic failure with dystonia and mental status changes
- PE: KF rings
- Dx: low ceruloplasmin, low copper in serum, increased copper in urine
- may have hemolytic anemia and abnl Calcium metabolism
- Tx: chelation with Penacillamine, low Cu diet, liver Tx
A
Wilson Disease
5
Q
- AR dz due to a defect in DNA processing and repairs
- CNS effects, skin, and eye findings, and immunologic sequelae
- hematologic malignancy, abnl blood values, ataxic gait, and oculocutaneous lesions
A
Ataxia Telangectasia
6
Q
- AR dz that accounts for half the cases of inherited ataxia
- presents in late childhood or early adolescence with a slow and clumsy gait
- elevated plantar arch, absent DTRs of LE, diabetes, cardiomyopathy leading to CHF
- Tx: supportive
A
Friedrich Ataxia
7
Q
- -occurs a few months after a strep infx
- elevated ASO titers
- quick random jerky movements and fidgety
A
Syndenham Chorea
8
Q
- chorea, hypotonia, and emotional lability
- AD dz with dementia
- Sx not apparent until adulthood
- younger children present with rigidity
A
Huntington chorea
9
Q
- present as eye blinking and movements of the head, face, or shoulders
- not caused by anxiety
- resolve on their own
- no intervention needed
A
Simple Motor Tics
10
Q
- tic disorder that is present for at lease a year
- may be associated with prolonged use of stimulant meds or OCD
A
Tourette Syndrome
11
Q
- ataxia, gait imbalance, incoordination, neck pain, HA, and increased ICP, afebrile
- CT or MRI is indiacted
A
Posterior Fossa Tumor
12
Q
- tumor that presents with pituitary and hypothalamic problems
- visual defects, neck pain, and HA, afebrile
- growth retardation, skull film with calcification in the sella tursica
- supratentorial
- Tx: surgery and XRAD
A
Craniopharyngioma
13
Q
- increased ICP of unknown etiology
- papilledema and eventual optic disc atrophy and blindness
- double vision, headache, and tinnitus
- causes: excess Vit A, steroids, thyroxine, lithium, tetracyclines
- Dx: MRI, LP
- Tx: LP, Diamox
A
Pseudotumor cerebri
14
Q
- progressive motor weakness and areflexia
- leg weakness and/or unsteady gait and pain
- starts in the lower extremity and progresses upwards in a symmetric fashion (ascending)
- difficulty rising from a sitting position or cannot shrug their shoulders
- preceeded by a viral illness or camphylobacter
- may be cranial nerve findings and dysautonomia
- Dx: incr protein in the CSF
- risk for resp failure: follow vital capacity, NIFs, or PFTs, not SpO2
- Tx: supportive, IVIG, or plasmapheresis
A
Guillan Barre
15
Q
- autoimmune disorder in which patient develops Ab against the Ach receptor in the NMJ
- weakness gets worse with activity and improves with rest
- ptosis
- associated with thymoma
- Dx: tensilon test, anti-Smith Ab, EMG
- Tx: pyridostigmine (acethylcholinesterase inhibitor), plasmapheresis, prednisone, thymectomy
A
Myasthenia Gravis
16
Q
- myasthenia gravis due to a genetic defect in the NMJ
- lifelong disease
- involves the eyes
- responds to Tensilon
A
Congenital Myasthenia Gravis
17
Q
- mysasthenia gravis due to mother’s Ab that crossed the placenta
- resolved within 6 months
- does not involve the eyes
- responds to Tensilon
A
Transient Myasthenia Gravis
18
Q
- infant with a weak cry, constipation, listlessness, hypotonia, and poor feeding
- descending paralysis, rapid onset
- unpasteurized honey
- toxin blocks the release of Ach from the NMJ
- Tx: supportive
A
Infantile Botulism
19
Q
- presents as proximal muscle weakness and butterfly malar rash on face, Gottron’s papules
- elevated CPK
A
Dermatomyositis
20
Q
- X-linked recessive (boys only)
- significant spontaneous mutation rate (mother is not always a carrier)
- absence of dystrophin
- poor head control in infancy and weakness in the hip muscles, lordotic posture, Gower sign, pseudohypertrophied calves, mild MR
- Dx: muscle Bx, elevated CPK, XP21 genetic testing
- cardiomyopathy and heart failure, resp failure and pna are frequent causes of death
- absence of tongue fasiculations and eye muscle involvement
A
Duchenne Muscular Dystrophy
21
Q
- affects both striated and smooth muscle (skeletal, heart, and GI)
- Autosomal Dominant
- presents at 4-5 yo with muscles that have slow relaxation after contraction (myotonia)
- endocrine problems, distal muscle wasting
- Dx: muscle Bx, CK may be normal
A
Myotonic Muscular Dystrophy
22
Q
- spinal pain, paresthesias, weakness, paralysis
- fevers, localized back pain, decreased anal tone, reduced sensation in the LE, increased reflexes
- direct extension from paraspinal tissues or seeding from a distant infection, IV drug use
- Dx: urgent spinal imaging
- Tx: Abx with staph coverage and emergent surgical decompression
A
Epidural Abscess
23
Q
-pt with evidence of a space occupying CNS lesion presenting with seemingly irrelevant URI findings consistent with chronic sinusitis
A
Brain Abscess
24
Q
- post-infectious myelitis
- due to lymphocytic infiltration and demyelination
- fever and a sudden onset of paralysis, followed by increased tone and hyperreflexia
- bowel and bladder dysfunction
- CSF with increases polys and negative gram stain
- pain on palpation of the spinal canal
- MRI shows cord swelling
- spontaneous recovery after a few months
A
Transverse Myelitis
25
- SMA Type 1
- degeneration of anterior horn cells, only motor function is affected, no sensory deficits
- presents in infancy with severe hypotonia and weakness, poor suck and tongue fasciculations*
- Dx: muscle Bx
Werdnig-Hoffmann Disease
26
- lipoma, patch of hair, hemangioma, discoloration or sacral dimple
- leg length discrepancy, elevated arches, and gait abnormalities
- diminished sensation in the perineal area, urinary and stool incontinence, UTIs
- Dx: MRI spine
Occult spinal dysraphism
27
- protrusion of the spinal cord and surrounding membranes though a defect in the vertebral column
- early orthopedic and urological eval
Myelomeningocele
28
-treatment for high index of suspicion for spinal trauma
Methyprednisolone 30mg/kg
29
-seizures that occur within the first month of life and have a variety of causes from birth defects to perinatal hypoxia to drug effects
Neonatal Seizures
30
- seizures that occur during a rapid rise or decrease in temperature
- occur in 2-5% of all children
Febrile seizure
31
- focal seizures that involve only part of the brain
- simple have a localized motor component limited to one part of the body, can progress to other parts of the body, children remain awake
- complex involve motor activity but child is unaware of what he or she is doing, post-ictal state present
Partial seizures
32
- short episodes in which the child stares into space with no awareness of the environment
- no post ictal period
- shorter than complex partial seizures
- end abruptly
- can be induced by hyperventilation
- 3Hz spike and wave
- AKA petit mal seizure
- Tx: ethosuximide or VPA
Absence seizure
33
- seizures that present between 4 and 6 months
- jack knifing, sudden flexion or extension of the body
- associated with mental retardation and have a poor prognosis
- hypsarrythmia
- Tx: Sabril, ACTH
- prognosis depends on developmental status prior to the onset of the seizures
- association with tuberous sclerosis
Infantile Spasms
34
- seizure activity more than 30 minutes in duration
- repeated seizures without a return to normal in between
- Tx: ABCs, dextrose, ativan. fosphenytoin
Status epilepticus
35
- have an aura or prodrome, minutes, hours, or days before the seizure
- LOC, bowel and bladder dysfunction, full body tonic clonic movements
- tonic phase: flexion of the trunk and extension of the back, arms, and legs, lasting
Generalized Tonic Clonic seizures
36
-potential side effect of Lamictal
Stevens Johnson Syndrome
37
- single or repetitive contractions of an isolated group of muscles
- loss of muscle tone and sudden brief shock like muscle twitches with the child falling forward
- no impairment of consciousness or memory
Myoclonic Seizures
38
- MC form of epilepsy in childhood
- self-limited seizures that involve the face and unilateral sensory involvement
- conscious but cannot talk
- often occur at night while asleep
- associated with migraines
- Tx: carbamazepine
- Dominant trait
Benign Rolandic Epilepsy
39
- may bathe or swim but not alone
- may drive if on meds and seizure free for 12 months
- may DC meds if seizure free for 2 years (unless juvenile myoclonic epilepsy)
Seizure Precautions
40
- static encephalopathy characterized by gross and fine motor abnormalities evident early in life
- 10% due to birth asphyxia
- perinatal infection is the MC risk factor
- risk decreased by maternal preeclampsia
Cerebral Palsy
41
- bilateral spasticity of the legs
- seen in preemies
- walking is delayed
- tip-toe walking
- excellent prognosis with normal cognitive function without seizures
Spastic Diplegia
42
- weakness of the upper extremities
- walking is delayed until age 2
- 1/4 are cognitively impaired
Spastic hemiplegia
43
- increased tone in all extremities
- lower > upper
- more severe
- mental retardation
- seizures, feeding difficulties, speech and visual difficulties
Spastic quadriplegia
44
-similar to spastic quadriplegia + dystonia and strange movements
athetoid cerebral palsy
45
- afebrile child with a HA, neck pain who then develops focal paralysis
- confirm with CT then CTA
- elevated lactate level and sensorineural deafness (mitochondrial association)
CVA
46
- acute onset of self-limited vertigo in the absence of vomiting or LOC
- nystagmus and pallor
Benign Paroxysmal Vertigo
47
-vertigo lasting for days associated with hearing loss
labrynthitis
