Neuro Flashcards
Breath holding attack
Toddlers with temper
Cry, hold breath, hypoxia, cyanosis, may briefly lose consciousness
Behavior modification therapy and distraction
Myoclonic seizures
Brief, oft repetitive, jerking movements of limbs, neck, or trunk
Generalized seizures
Always LOC, no warning, symmetrical seizure, B/L synchronous activity on EEG or varying asymmetry
Absence seizures
Transient LOC, abrupt onset and termination
No motor phenomena except flickering eyelids or minor alteration in tone
Can be precipitated by hyperventilation
Few seconds, multiple, often not doing well at school. “Dazing out”, “daydreaming”
Tonic clonic seizures
Rhythmic muscle contractions following tonic phase. May fall to ground during tonic phase, do not breathe. May become cyanosed.
Clonic phase has irregular breathing, cyanosis may persist, saliva may accumulate.
Tongue biting, urinary incontinence may be a feature
West syndrome
4-6mo.
“Salaam spasms”: violent flexing of neck followed by hyperextension shoulders (watch a youtube video–easier to see than explain)
1-2s oft in multiple bursts of 20-30, often on waking
May occur throughout day
Social interaction often deteriorates
Tx-West Syndrome
Vigabatrin, steroids
Note “gaba” in vigabatrin…helps remember MOA :)
Lennox-Gastaut syndrome
1-3 years. Multiple seizure types, mostly drop attacks, tonic clonic, and atypical.
Neurodevelopmental arrest or regression and behavior disorder
Benign epilepsy with centrotemporal spikes
4-10 years
Tonic clonic seizures in sleep or simple focal seizures with awareness abnormal feelings in tongue and distortion in face
15% childhood epilepsies
Does not always require treatment-almost all remit in adolescence
Juvenile myoclonic epilepsy
Adolescence-adulthood
Myoclonics but generalized tonic-clonic and absences may occur.
Usu after waking
Typical hx of throwing drinks and cornflakes
First line treatment for all generalized seizures
Valproate
Can also use ethosuximide for absence
Second line for all generalized seizures
Lamotrigine
First line for focal seizures
Carbamazepine, valproate.
Lamotrigine good efficacy, but titrate slowly
Side effects carbamazepine
Rash, neutropenia, hyponatremia, ataxia
Side effects valproate
Weight gain, hair loss
Rare idiosyncratic liver failure
Management status epilepticus
- Call for help
- ABC
- Glucose
- If vasc access: 0.1mg/kg lorazepam. Repeat if not working
No vasc access: 0.5mg/kg buccal midazolam or 0.5mg/kg PR diazepam. - Paraldehyde 0.4mL/kg PR
If no response, SENIOR HELP - Phenytoin 18mg/kg IV over 20min or phenobarb 15mg/kg if already on oral phenytoin
Reflex anoxic seizures
Infants/toddlers
Pain or discomfort (e.g. minor head trauma, fever, fright, cold food) causes them to go pale, then lose consciousness. May have tonic clonic.
Due to cardiac asystole from vagal inhibition
Spontaneous resolution
Febrile convulsions
6mo-6years
Rapid increase in temperature (not prevented by antipyretics)
3% kids
If complex, increases risk subsequent epilepsy.
30-40% chance will have another
Ix: BM, cause of fever, rule out meningitis
Noncommunicating hydrocephalus
Obstructive lesion
Communicating hydrocephalus
Problem of arachnoid villi absorbing CSF
Causes non-communicating hydrocephalus
Congenital: aqueductal stenosis, Dandy Walker, Arnold Chiari
Posterior fossa neoplasm or vascular malformation
Intraventricular hemorrhage in preterm
Treatment hydrocephalus
Ventricular shunt
Key feature neuromuscular diseases
Weakness
Presentation of anterior horn cell problems
Weakness, loss of reflexes, fasciculation, wasting
Diseases that affect anterior horn cells
Polio
SMA
ALS
Presentation neuropathy
Often distal nerves
Weakness, loss of reflexes, sensory loss, temperature change
presentation myopathy
Weakness, wasting, changes in gait
Presentation of neuromuscular junction disorders
Worsening of weakness throughout day
Ix for myopathy
CK (increased in Duchenne's, Becker's) Muscle biopsy USS muscles MRI muscles DNA testing
Ix-neuropathy
Nerve conduction studies
DNA testing
EMG differentiates myopathy vs neuropathy
Spinal muscular atrophy (SMA)
AR degeneration of anterior horn cells
Type I=Werdnig Hoffman (worst type). Death from resp failure by age 1
Presents as floppy baby
Hereditary motor sensory neuropathies
E.g. Charcot-Marie-Tooth
Demyelinating neuropathy
Nerves may be hypertrophic due to attempts at remyelinization
Onion bulb on nerve biopsy
Distal atrophy
Pes cavus
Rarely distal sensory loss and decreased reflexes
Guillain Barre Syndrome
Acute immune polyradiculopathy
Ascending symmetrical weakness, loss reflexes, and autonomic involvement
Sensory sxs less striking than paresis but can be initial sxs and unpleasant
Bulbar involvement can cause unsafe swallow
25% have resp muscle involvement
Supportive tx, esp resp support
95% fully recover but may take up to 2y
Bell’s palsy
LMN problem, CN VII
Probably post-infectious (HSV)
Roids in 1st week may decrease edema
Main complication is conjunctival infection (incomplete eye closure/blinking)–eye patch
If both CN 7 and 8—think compressive lesion at cerebellopontine angle
If vesicles and CN 7, give acyclovir
Associated with coarc, so take BP both arms
If B/L suspect sarcoid, Lyme
Recovery complete in most, but may take several months
Tension headache
Band of pressure/tightness, “vice” around head
Gradual onset
Migraine
Paroxysmal, often unilateral
Throbbing
GI disturbances-N&V, abdo pain common, visual problems
Sleep often relieves
90% without aura; 10% with
1-2% complicated–other neuro signs, altered sensation
Causes acute headache
Febrile illness Migraine Stress Acute sinusitis Meningitis/encephalitis Head injury Subarachnoid hem, intracerebral hem Benign intracranial hypertension Drugs (EtOH, solvents) Others, e.g. ice cream
Headache sxs due to space occupying lesion
Worse on lying down
Morning vomiting
Night time awakening
Mood change, personality, or educational performance change.
Others” visual field defects, CN abnormalities, abn gait, torticollis, growth failure, papilledema, cranial bruits
Tx-headaches
Psych support if needed Relaxation techniques Analgesia (NSAIDs) Anti-emetics (prochlorperazine, metoclopramide) Intranasal sumatriptan
Temporal lobe seizures-prodrome
May have aura with taste/smell abnormalities and distortions in sound/shape.
Lip smacking, plucking clothing, automatisms
Parietal lobe seizures
C/L dysasthesias (altered sensation) or distorted body image
Ataxia-definition
Incoordination movement, speech, and posture
Can be due to cerebellar or posterior sensory problems
Causes cerebellar ataxia
Medication/drugs (EtOH, solvents)
Post-viral (varicella)
Posterior fossa lesions (medulloblastoma)
Genetic disorders (Friedreich’s ataxia, ataxic CP, Ataxia-Telangiectasia)
Friedreich’s ataxia
AR tri-nucleotide repeat in frataxin gene
Worsening ataxia, distal leg wasting, absent lower limb reflexes, pes cavus, dysarthria
Evolving kyphoscoliosis and cardiomyopathy can cause cardiorespiratory death at 40-50 years
Ataxia Telangiectasia
AR disorder in DNA repair. ATM gene
Motor delay in infancy, oculomotor problems with incoordination and delay in ocular pursuit objects
Difficult balance and coordination by school age
Deterioration…dystonia and cerebellar signs
Telangiectasias in conjunctive, neck,, shoulder
Increased infections (IgA defect)
ALL in 10%
Increased white cell sensitivity to irradiation
Causes stroke in childhood
Cardiac: congenital heart disease, e.g. TOF
Heme: sickle cell, deficient anti-thrombotic factors (e.g. Protein C)
Inflamm/AI: SLE
Metabolic: homocystinuria, mitochondrial (MELAS)
Vascular: moyamoya
Post-infection, e.g. varicella
NTDs
Failure fusion neural plate in first 28 days.
Includes anencephaly, spina bifida occulta, meningocele, myelomeningocele
Anencephaly
Failuere most cranium and brain to develop
Spina bifida occulta
Failure fusion vertebral arches. May have overlying skin lesion, e.g. tuft of hair
Encephalocele
Extrusion brain and meninges from midline skull defect
Meningocele
meninges herniate.
Good prognosis with sx
Myelomeningocele
Can cause wide range problems, e.g. paralysis legs, muscular imbalance leading to hip dislocation, sensory loss, bladder and/or bowel denervation, scoliosis, hydrocephalus (Arnold Chiari)
Juvenile myasthenia
Ab against AChR on post junctional synaptic membrane
After 10 years age
Ophthalmoplegia, ptosis, loss facial expression, difficulty chewing
Generalized (esp proximal) muscle weakness
Tx: anticholinesterases, e.g. neostigmine, pyridostigmine
Pred, azathioprine
Plasma exchange if severe
Duchenne’s muscular dystrophy
XR. Deletion dystrophin gene
1/4000 male infants
Present with waddling gait or language delay. Mount stairs one at time
Calf pseudohypertrophy, no longer ambulant by 10-14
Death by late teens-20s (resp failure, cardiomyopathy)
1/3 have learning disability
Scoliosis common
Increased CPK
Treatment-Duchenne’s
Appropriate exercise. Prevent ankle contractures with passive stretching and night splints Orthoses CPAP at night if nocturnal hypoxia Steroids 10d/month
Becker’s muscular dystrophy
Truncated dystrophin gene
Similar to Duchenne’s but slower progression
Benign acute myositis
Post-viral (URTI)
Pain and weakness
Increased CPK
Dystrophia myotonica
Trinucleotide expansion
newborns: hypotonia, feeding/resp difficulties
Older kids: myopathic facies, LD, myotonia
Adults: cataracts. Males have baldness and testicular atrophy
Death from cardiomyopathy
Causes of floppy baby
Hypoxic-ischemic encephalopathy Cortical malformations Downs Prader Willi Hypothyroidism, hypocalcemia SMA, myopathy,myotonia, MG
NFI
1/3000 AD. Highly penetrant Cafe-au-lait spots Axillary freckling Optic gliomas Lisch nodules (slit lamp examination) Bony lesions from sphenoid dysplasia
NF2
Less common than NF1
presents in adolescence
B/L acoustic neuromas–deafness, possibly cerebellopontine angle syndrome
Tuberous sclerosis
AD Ash leaf spots Shagreen patches (rough patches skin, usu lumbar spine) Adenoma sebaceum (angiofibroma)-butterfly distribution Infantile spasms, devel delay Epilepsy, oft focal Oft autistic features when older Rhabdomyomata in heart Polycystic kidneys
Sturge Weber
Port wine stain (“hemangiomatous facial lesion”) in trigeminal nerve distribution (always involving opthalmic division)
Railroad track calcifications (gyri) on skull CT
In most severe form-epilepsy, LD, hemiplegia
Tay Sachs
Defect in hexosaminidase A AR. Ashkenazi Jews Cherry spot on macula, devel regression, exaggerated startle response Severe hypotonia, enlarging head Death 2-5yrs Prenatal detection possible
Gaucher’s
Defect in Beta-glucosidase
Ashkenazi Jews
Splenomegaly, bone marrow suppression, bone involvement
Normal IQ
Niemann Pick
Defect in sphingomyelinase
At 3-4months: feeding difficulty, FTT, HSM, devel delay, hypotonia, decreased hearing and vision
Cherry red spot on macula (50%)
Death by age 4
Subacute sclerosing panencephalitis
Complication of past measles infection
Neuro regression in adolescence, characteristic EEG
