Neurology Flashcards
Diagnosis criteria and treatment of Tourette’s syndrome
- Motor tics typically start after age 5-6
- If tic has been present for > 1 year, diagnosis can be made of Tourette’s
- Treatment is mostly supportive but if tic is particularly bothersome can use haloperidol or pimozide
Acute arterial ischemic stroke causes/symptoms
- Embolic causes: artery to artery embolism from carotid/vertebral dissection, intracardiac thrombi, paradoxical embolism from venous system through cardiac shunt
- In situ thrombosis causes: hypercoagulable states
- Vasculopathy causes: focal cerebral arteriopathy from post-infectious causes
- Genetic causes: Fabry disease, a-galactosidase A deficiency
- Metabolic causes: MELAS
- S/sx: acute focal neurologic deficit
Acute arterial ischemic stroke diagnosis, management, complications
- Diagnosis: start with CT, then dig further with CTA/MRA
- Complications: acute stroke in posterior fossa can lead to edema and obstructive hydrocephalus
Pseudotumor cerebri signs/symptoms
- Headaches worse when laying flat, N/V, transient vision loss, obesity, females
- CN VI palsy (double vision) and papilledema on exam
- Complication: optic disc atrophy and blindness if not treated
Atlantoaxial instability complications
- Spinal cord compression
- Hyperreflexia and increased tone in lower extremities
- T21 kids
Benign rolandic epilepsy symptoms
- Rhythmic twitching of one side of the face and ipsilateral arm with a lot of drooling
- Seizures are usually infrequent and often occur during sleep
- Can be associated with migraines
- If they happen while awake the child is fully aware but unable to speak
- Resolve by 16-18 years of age
- EEG with biphasic focal centroemporal spikes and slow waves
- Don’t need meds unless 3 or more seizures
Number one cause of death among all childhood cancers
Brain tumors
Symptoms of infratentorial/posterior fossa brain tumor
- Headache, vomiting (especially in the morning), ataxia in an afebrile child
- Can have head tilt, torticollis, decreased upward gaze (CN VI dysfunction)
- Papilledema
- Often < 6 years of age
Medulloblastoma imaging
- Occurs midline (commonly in IV ventricle)
- Contrast enhancing, can cause hydrocephalus
Supratentorial mass symptoms
- Often have motor findings (weakness, seizures, etc)
- Commonly > 8 years old
Types of infratentorial masses
- Pilocytic astrocytoma
- Medulloblastoma
- Ependymoma
- Brain stem glioma
- Atypical teratoid/rhabdoid
Types of supratentorial masses
- Craniopharyngioma
- Diencephalic
- Germ cell tumors
Craniopharyngioma symptoms and imaging
- Sits right above the optic chias –> personality changes, headaches, vision changes (superior temporal field cut), growth failure
- Benign but can recur
- Often in ages 5-10
- Imaging shows calcification in the sella turcica
Optic nerve glioma association
Neurofibromatosis
Brain tumor treatment
- Cut out the tumor (need diagnosis)
- Radiation and chemo after
Chiari II malformation
- Cerebellar tonsils and vermis descend through foramen magnum
- Almost always associated with meningomyelocele
- Often have hydrocephalus
- Prenatal diagnosis, often need surgical repair
Chiari I malformation
- Cerebellar tonsils through foramen magnum
- Often asymptomatic
- Often don’t require surgery
Chairi III malformation
- Chiari II + occipital encephalocele
- Developmental delay, hydrocephalus, seizures
- Often don’t have good prognosis even after surgical repair
Holoprosencephaly
- Single primitive ventricle
- Fused thalami
- Absent olfactory bulbs/tracts and optic tracts
- Midface underdevelopment (single eye, rudimentary nose, single nostril nose, ocular hypotelorism, cleft lip/palate)
Holoprosencephaly genetic/exposure associations
- Trisomy 13, 15, 18
- Maternal diabetes
- In utero alcohol exposure
Teenager with frontal/band like pressure headaches
- Stress/tension headache
- Tx: eliminate stressor, treat depression
Lissencephaly
- Abnormal neuronal migration at 9-13 weeks
- Paucity of white matter
- Severe intellectual disability, epilepsy, hypotonia
- Associated with Fukayama, Walker Warburg
Cerebral Palsy definition
- Static encephalopathy (single perinatal CNS insult during early development)
- Chronic, non-progressive motor deficit
Spastic types of CP
- MC type of CP overall
- Quadriplegia
- Diplegia: most common in the lower extremities (tip toe walking or delayed walking), most common in preemies (IVH)
- Hemiplegia: more common in the upper extremities, have early handedness, focal lesions
Dyskinetic CP association
- Kernicterus
- HIE
Most common risk factor for premature babies to develop CP
Perinatal infections
Dyskinetic CP
Spastic quad symptoms but also have dystonia and strange movements
Migraine headache symptoms
- Episodic
- Cause kids to stop activities and go lay down
- Light/sound sensitivity
- Nausea/vomiting
- Hemiparesis, temporary visual deficits
Migraine headache abortive treatment
- Ibuprofen, tylenol (no more than 3 days per week)
- Fluids, rest
- Ergotamines
- Sumatriptan
Management of CP
- Seizure management
- PT/OT/ST
- Ortho issues over time
- Baclofen pump
- Botox
Migraine headache prophylactic treatment
- Cyproheptadine
- Topiramate
Causes of pediatric stroke
- Embolic (1/4th from the heart)
- Dissection (trauma, can be minor)
- Vasculitis
- Sickle cell
- Coagulopathies
- Metabolic (MELAS, Fabry, Homocystinuria)
- CVA malformations (commonly cause hemorrhagic strokes)
- NAT
Stroke workup
- MRI, MRA
- Echo/EKG/Tele
- Urine drug screen
- Labs: CBC, sed rate, PT, PTT, antiphospholipid antibodies, factor V leiden, prothrombin mutation, lactate, protein C/S, homocystine, antithrombin III
Stroke management
- tPA if within first 3 hours and no bleeding
- baby aspirin otherwise
Indications for head CT, MRI, ultrasound
- CT: hemorrhage, tumors, abscesses
- MRI: partial seizures, herpes encephalitis
- Ultrasound: accelerating head circumference in infants
Clues to pseudoseizures
- Absence of post-ictal state
- Often in daytime with other people around
Paroxysmal non-epileptic event
Looks like a seizure but has no EEG changes
Common causes of metabolic seizures
Hyponatremia, hypoglycemia, hypocalcemia, urea cycle disorders, pyridoxine deficiency
Febrile seizure facts
- Age 6 months to 5 years
- 3-5% of kids will have a febrile seizure sometime in their life
- Associated with no other seizure history
- 1/4 of kids have a family history of febrile seizures
Facts for complex febrile seizures
- > 15 minutes
- Recurs within 24 hours
- Focal onset
Recurrence of febrile seizures
- 1/3 of kids will have a second febrile seizure
- Risk factors for recurrence: young age, low fever at time of first seizure, family history of febrile seizures
- 5% of patients will go on to develop epilepsy (increased risk with FH or complex seizure)
Medications that can cause seizures
- Chemo: cyclosporine, IT methotrexate
- Isoniazid
- Insulin
- Bupropion
- Theophylline
- Cocaine
Indications for LP in febrile seizure
If complex partial seizures or febrile status epilepticus or if baby was partially treated with antibiotics
Symptoms of absence seizures
- Brief staring spells (< 30 seconds), not responsive during it
- No postictal period but don’t remember the event
- Spacing out/blinking
- Triggered with hyperventilation
EEG finding and treatment for absence seizures
- 3 second spike and wave
- Tx: ethosuximide
Side effects of valproic acid
- Thrombocytopenia
- Elevated LFTs and lipase
Phases of generalized tonic clonic seizure
- Tonic: flexion of trunk, extension of back/arms/legs that usually lasts about 30 seconds
- Clonic: convulsion movements, can be apneic during this, also time of bladder/bowel loss
- Postictal: unconscious then sleepy/confused, can’t remember the seizure
Side effect of lamotrigene
Stevens Johnson Syndrome
Symptoms of juvenile myoclonic epilepsy
- Myoclonic jerks upon awakening (often occur in adolesence)
- Generalized tonic clonic seizures in most of patients
- Absence seizures in 1/3 patients
- Precipitators: lack of sleep, emotional stress, alcohol
- Tx: valproic acid or levetiracetam
Infantile spasms, hypsarrhythmia, developmental delay
West Syndrome
Symptoms of infantile symptoms
- Present between 3-9 months
- Spasms in clusters: sudden flexion or extension of the body
- Often have severe developmental delay
Infantile spasm genetic association
Tuberous sclerosis
Treatment of infantile spasms
- ACTH or steroids
- If developmental delay before onset of seizures –> poor prognosis
- After infantile spasms, they often develop other seizure types
Simple partial seizure symptoms
- Consciousness is maintained
- Focal abnormal movement (usually arms or face)
- Often occur as patient falls asleep or just wakes up
- Can be sensory or autonomic seizures
Treatment of simple partial seizures
- Carbamazepine often used
- Many cases remit during adolescence and treatment is not always necessary
Complex partial seizure symptoms
- Alteration in consciousness
- Often have facial movements or lip smacking
- Can appear to be responding to auditory or visual hallucinations
- Need MRI and EEG
Definition of status epilepticus
- > 30 minutes
- Check a glucose
- Give ativan then fosphenytoin load
Symptoms of Bell’s Palsy
- LMN of CN VII palsy
- Loss of ALL muscles in forehead and lower part of the face
- Associated with Lyme’s disease, hypertension, otitis media, and ALL
Contraindications to LP
- Symptoms of increased ICP
- Focal neuro signs
- History of coagulopathy
- Cardiorespiratory instability
Medications that can cause pseudtumor cerebri
- High dose vitamin A (Retin A)
- Steroids
- Thyroxine
- Lithium
Pseudotumor cerebri treatment
- Acetazolamide (carbonic anhydrase inhibitor)
- Shunt placement if severe
- LP can be therapeutic
Cushing’s triad
- Hypertension
- Bradycardia
- Abnormal respirations
- Impending brain herniation
Unilateral pupil dilation
Uncal herniation (due to compression of oculomotor cranial nerve)
Cranial bruit in a neonate with hydrocephalus and a history of heart failure
Vein of Galen malformation
Symptoms of narcolepsy
- Excessive daytime sleepiness and sudden sleep attacks
- Cataplexy, sleep paralysis, hypnagogic hallucinations
- Onset in adolesence
- Dx with sleep study
- Tx with behavioral/environmental changes, may need meds
Causes of ataxia
- Toxins (ethanol, pesticides)
- Infection (postviral or Guillain Barre)
- Neoplasm (glioma, medulloblastoma)
- Other meds: anticonvulsants, alcohol, thallium
Symptoms of ataxia telangeictasia
- Ataxia
- Telangeictasias in skin and eye
- Frequent upper/lower respiratory infections (IgA, IgG, T cell dysfunction)
- Intellectual disability
- High incidence of malignancy (Hodgkin lymphoma and leukemia)
- Autosomal recessive
Friedreich Ataxia symptoms
- 50% of all causes of inherited ataxia
- Autosomal recessive
- Late childhood/early adolesence
- Slow/clumsy gait (elevated plantar arch)
- Can also have decreased strength and reflexes in lower extremities
- Risk for cariomyopathy
Epidural vs subdural hematoma
- Epidural: between blood and skull bone, lens shape, can have lucid period, need to act fast (can herinate quickly)
- Subdural: between brain parenchyma and dura, linear and more diffuse distribution
Huntington chorea triad
- Chorea
- Hypotonia
- Emotional lability
Autosomal dominant
Tx of chorea: dopamine blocking agents (haloperidol), risperidone
Associations with acute dystonic reaction
- Sx: neck hyperextension and decreased extraocular movements
- Can be caused by neuroleptics (like metoclopramide or promethazine)
- Tx with benadryl
Type of medication that can unmask tic disorder
- Stimulants (don’t cause tics but can unmask them)
- Tics are repetitive movements that improve or disappear during purposeful movements and can by suppressed
Symptoms of an epidural abscess
- Spinal pain, paresthesias, weakness, paralysis, fevers
- Localized spinal compression: decreased anal tone, reduced sensation in lower extremities, increased reflexes
- Risk factor: IV drug users
- Tx: MRI/CT scan, antistaph antibiotics, surgical decompression
Child with a fever with abrupt onset of weakness, hypotonia, and decreased reflexes followed by increased tone and hyperreflexia
- Acute transverse myelitis
- Follows an infection and is due to lymphocytic infiltration and demyelination of nerves due to inflammation
- CSF has increased neutrophils, negative gram stain
- Pain on palpation of the spinal canal and MRI shows cord swelling
- Tx: spontaneous recovery in a few months
Treatment for extrinsic spinal cord mass lesion
High dose IV dexamethasone
Symptoms and biology of spinal muscle atrophy type 1 (Werdnig-Hoffman disease)
- Presents in infancy as severe hypotonia and weakness (poor suck, tongue fasciculations)
- Degeneration of anterior horn cells (ONLY MOTOR FUNCTION AFFECTED)
- Autosomal recessive
- Dx with muscle biopsy
When do NTDs occur
- Between 3rd and 4th weeks of gestation
- Risk factors: maternal folate deficiency, maternal anticonvulsant use
Symptoms of occult spinal dysraphism
- Lipoma, patch of hair, hemangioma, discoloration, sacral dimple
- Later symptoms: leg strength discrepancy, elevated arches, gait abnormalities, sensory issues, incontinence
- Need MRI
Treatment for spinal trauma
- Methylprednisone (not dex like spinal cord compression), immobilizing cervical and lower spine
Nerve and symptoms involved in bell’s palsy
- CN 7 (facial nerve)
- Unilateral facial paralysis that develops abruptly –> forehead weakness (decreased wrinklingon teh affected side)
- Tx: prednisone for 5 days
Ramsay hunt facial paralysis
- Facial paralysis with painful vesicles in the ear canal
- Due to reactivation of variella zoster virus
- Tx: valacyclovir
Ascending progressive motor weakness and arflexia
Guillain barre syndrome
- ALWAYS have areflexia
- Often preceded by vial illness or campylobacter
- Can also have CN findings and dysautonomia on exam
Labs and treatment for guillain barre
- CSF with increased protein, normal cell count
- Measure pulmonary function tests (NIF) –> respiratory failure is biggest issue
- Tx: plasmapheresis, IVIG (NO STEROIDS)
Cause of myasthenia gravis
- Autoimmune disorder with development of antibodies against acetylcholine recpetor in neuromuscular junction
Symptoms of myasthenia gravis
- Weakness that gets worse with activity and improves with rest (more tired as the day goes on)
- PTOSIS!!
- Can be associated with thymoma
Workup and treatment for myasthenia gravis
- Tensilon test
- Anti-aceytlcholine recpetor antibodies
- EMG
- Tx: pyridostigmine (inhibits acetylcholinesterases), plasmaphresis, plasma exchange
- Thymectomy can be curative in kids
Becker muscular dystrophy
- Muscle weakness that is milder with slower deterioraiton and later onset than Duchenne
- Can have severe cramps and more severe cardiac involvement
Duchenne muscular dystrophy genetics
- X linked recessive (Xp21 gene testing)
- Absence of dsytrophin
- Dx with muscle biopsy
Duchenne muscular dystrophy symptoms
- Poor head control in infancy
- Weakness in hip muscles or lordotic posture in toddlers, can’t walk up stairs
- Gower sign: walks hands up his legs in order to stand up
- Pseudohypetrophy of calves: fat deposition and prolifeartion of collagen
- Mild intellectual disability
- Elevated CPK (can be elevated before muscle weakness starts)
- ABSENCE of tongue fasciculations and eye muscle involvement
- Complications: cardiomyopathy, respiratory failure, pneumonia
Myotonic muscular dystrophy symptoms
- Affects skeletal muscle, heart, and GI tract
- Presents around 4-5 years of age with muscles that have slow relaxation after contraction (esp in hand)
- Autosomal dominant
Definition of encephalocele
Sac like protrusion of the brain and membranes that cover it through an opening in the skull
Definition of spina bifida
Opening in vertebral column caused by the failure of the caudal neural tube to close
- A/w chiari II malformation and hydrocephalus
- Risk when mom usings valproate or carbamazepine
Nerves involved in Marcus Gunn phenomenon
- Trigeminal and oculomotor nerves
- Simultaneous eyelid blinking during sucking jaw movements
