Neurology Flashcards
1
Q
headache history
A
- history
- what is the typical course?
- how long does the headache last?
- what makes the headache better/worse?
- what precipitates the headache?
- what other symptoms do you have?
- how does this compare to past headaches?
2
Q
headache: review stress-related factors, red flags that prompt futher exploration
A
- review stress-related factors
- school difficulty
- family relationships
- peer relationships
- activity schedule
- sleep hygiene
- caffeine intake
- red flags that prompt further exploration
- dramatic increase in headache severity
- headache that awakens a child from sleep
- change in established headache pattern/disrupt school and daily activities
- gradually increasing frequency and severity
- suggests increasing intracranial pressure
3
Q
headach physical exam, red flags in history or exam may set off further investigation
A
- physical exam
- growth, head circumference, blood pressure
- intracranial hypertension (papilledema)
- focal neurologic signs
- general exam findings
- rhinitis, dental abscess, bruit, head trauma, hematoma,
- red flags in history or exam may set off further investigation
- head imaging (CT for hemorrhage, MRI for tumor & cerebellar imaging)
- electroencephalography (EEG)
- +/- sleep deprivation
4
Q
tension headache, cluster headache
A
- tension headache
- diffuse
- symmetric
- often related to fatigue
- cluster headache
- extreme deep pain in and around one eye
5
Q
migraine headache
A
- migraine
- triggered by stress, vomiting, family history
- classification
- migraine with aura (visual or otherwise)
- migraine without aura
- complicated migraine (transient focal abnormality)
6
Q
childhood migraine variants
A
- paroxysmal torticollis
- attacks of head tilt, sometimes with vertigo +/-vomiting
- requires ruling out posterior fossa pathology
- benign paroxysmal vertigo
- attacks of unsteadiness with nystagmus and vomiting followed by sleep
- may precede development of typical migraine
- requires ruling out epilepsy and CNS tumor
- cyclic vomiting
- protracted attacks of vomiting, 1-4 times/hour, up to 5 days
- requires ruling out epilepsy, GI disorder, urea cycle disorder
- abdominal migraine
- attacks of migraine lasting 1-72 hours, untreatable by other means
- midline, dull, moderate to severe pain
- associated with anorexia, nausea, vomiting and pallor
- diagnosed often by response to anti-migraine therapy
- requires ruling out other GI disorder
- confusional migraine
- episodic disorientation or combativeness, sometimes followed by headache
- requires ruling out drug abuse, epilepsy, CNS ischemia
7
Q
migraine therapies
A
- migraine acute episode
- sleep
- acute treatment
- acetaminophen, ibuprofen
- sumatriptan, other triptans
- rescue treatment
- NSAIDs
- promethazine
- metoclopramide
8
Q
migraine prophylaxis
A
- create management plan to prevent stressors
- biofeedback (stress reduction)
- physical modalities (massage, PT, exercise)
- medications (usually involves a neurologist)
- cyproheptadine
- antihypertensives
- propranolol, verapamil
- tricyclic antidepressants
- amitriptyline, nortriptyline
- anticonvulsants
- valproate, topiramate, gabapentin
9
Q
headache summary
A
- summary
- acutely rule out underlying pathology
- address exacerbating factors
- management plan
- medications (start with acetaminophen, ibuprofen)
- abortive agents
- daily prophylaxis (neurologist)
10
Q
pseudotumor cerebri
A
- increased intracranial pressure in the absence of identifiable intracranial mass or hydrocephalus
- postulated to be due to impaired CSF reabsorption
- risk factors
- obesity
- female
- sinus thrombosis
- head injury
- chronic CO2 retention
- systemic lupus erythematosus
- acute
- headache
- pulse synchronous tinnitus
- pain behind the eye
- pain with eye movements
- transient visual obscurations
- blurred vision or double vision
- CN VI paresis
- vomiting
- macrocephaly
- altered behavior
- chronic
- growth impairment
- optic atrophy
- visual field loss
- total blindness
- diagnosis (one of exclusion)
- CT
- rules out hydrocephalus
- MRI
- rules out intracranial mass
- rules out hydrocephalus
- ophthalmologic exam
- papilledema
- optic nerve changes
- lumbar puncture
- measurement of opening pressure
- normal CSF panel and culture
11
Q
pseudotumor cerebri treatment
A
- treatment of underlying causes
- weight loss is mainstay of therapy
- treat anemia
- medical treatment
- diuretics
- acetazolamide (Diamox) - carbonic anhydrase inhibitor
- glucocorticoids
- lumbar puncture
- diuretics
- surgical treatment
- optic nerve sheath decompression
- lumboperitoneal shunt
12
Q
seizures
A
- a sudden, transient disturbance of brain function, manifested by involuntary, motor, sensory, autonomic, or psychic phenomena, alone or in any combination often accompanied by alteration of loss of consciousness
- epilepsy
- repeated seizures without evident cause
- recurrent, unprovoked seizures
- classification
- symptomatic
- cause is identified or presumed
- idiopathic
- cause is unknown or presumed to be genetic
- symptomatic
- symptomatic (examples)
- infection (meningitis/encephalitis)
- trauma
- metabolic (hypoglycemia, hyponatremia)
- hypoxic
- tumor
- malformation (hydrocephalus)
13
Q
nonepileptic paroxysmal events
A
- benign nocturnal myoclonus
- shudder attacks
- tics & Tourette’s syndrome
- sleep orders
- night terrors
- sleepwalking/talking
- cataplexy, narcolepsy
- nightmares
- migraine
- benign paroxysmal vertigo
- paroxysmal torticollis
- conversion reaction & pseudoseizure
- gastroesophageal reflux
- masturbation
- hypoglycemia
- temper tantrums & breath-holding
- syncope and vasovagal events
- paroxysmal dystonia or choreoathetosis
14
Q
seizure exam, treatment, abortive meds
A
- history
- events prior (aura, what was person doing?)
- events during and after
- exam
- laboratories based on clinical suspicion
- CBC, metabolic abnormalities
- CT scan/MRI - indicated if seizure focus suspected
- EEG
- not sensitive and not specific
- may help classify seizure type to determine therapy
- may reveal subclinical seizures
- treatment
- ABCs
- most seizures are brief and ABCs are of utmost importance in brief seizures
- protect against self-injury, airway clearance, monitor ABCs
- anticonvulsants - if seizures are prolonged or are hindering ABCs
- education
- seizure precautions
- medication (side effect monitoring, compliance)
- daily life
- swimming
- adequate sleep
- driving
- pregnancy
- ABCs
- abortive medications
- benzodiazepines
- lorazepam parenterally
- diazepam orally or rectally
- midazolam nasally, orally, rectally
- When giving benzos, you need to be careful of respiratory depression
- phenobarbital and fosphenytoin
- benzodiazepines
- long-term anticonvulsants
- different drug classes used for different types of epilepsy
15
Q
febrile seizure
A
- convulsion associated with a temp > 38.0° Celsius
- child 6 months to 5 years of age
- Not caused by CNS illness or metabolic abnormality
- no history of previous afebrile seizures
- most common childhood seizure
- incidence 2-5% of children under 5 years of age
- simple febrile seizure – meets all criteria:
- duration under 15 minutes
- total duration less than 30 minutes if in series
- no recurrence in 24 hours
- no focal features
- duration under 15 minutes
- complex febrile seizure – meets one or more criteria:
- duration over 15 minutes
- recur in series for total duration > 30 minutes or more than seizure in a 24 hour period
- focal features
- 65-90% of all febrile seizures are simple type
- Inciting agent
- Viral infections
- immunization-related fever
- predisposing factors
- heredity - SCN1a mutations - Dravet, GEFS+
- maternal alcohol intake and smoking during pregnancy raises risk 2-fold
- degree of fever is widely variable
- height of fever does not seem to correlate with onset of seizure
- often the first sign of illness
- simple febrile seizures most commonly are generalized and tonic-clonic
- reassurance
- if fever work up unremarkable
- if neurologic exam unremarkable
- treat fever to treat fever
- treat underlying illness
- decreasing fever in febrile seizure patient does not decrease chance of another febrile seizure
- antiepileptic medication if in status (status epilepticus)
16
Q
febrile seizure differential diagnoss
A
- differential diagnosis
- chills/rigors because of fever
- meningitis
- encephalitis
- intracranial tumor
- metabolic disorder
- neurologic disorder (developmental delay)
17
Q
diagnostic evaluation
A
- diagnostic evaluation
- EEG usually not indicated for simple febrile seizure if history and physical benign
- neuroimaging red flags
- macrocephaly or microcephaly
- focal features of seizure or abnormal neurologic exam
- signs of increased intracranial pressure
- history of signs of trauma
- electrolytes, glucose, calcium, BUN should be measured if history and physical indicate (vomiting, diarrhea, edema, dehydration) or with complex febrile seizure
- Search for underlying cause of fever based on clinical suspicion
- AAP recommends stronger consideration of LP
- infants younger than 12 months who have had first seizure with fever
- febrile seizures occurring on or after second day of illness
18
Q
febrile seizure: prevalence of meningitis
A
- the prevalence of meningitis among patients with febrile seizures was 1-2%
- the absence of any remarkable findings on the history or physical examination makes bacterial meningitis unlikely as the cause of the fever and seizure
19
Q
febrile seizures ABCs
A
- ABCs
- anticonvulsants
- if seizure is prolonged (>10 minutes)
- watch for respiratory compromise
- drugs in office or ED
- benzodiazepine (midazolam, lorazepam)
- fosphenytoin
- rectal diazepam (option for home use)
20
Q
febrile seizures role of preventative tx
A
- role of preventive therapy
- antipyresis
- around-the-clock use not shown to be of benefit to all children with febrile seizures
- unknown if some subset benefits
- intermittent use warranted
- anticonvulsants
- “based on the risk and benefits of effective therapies, neither continuous nor intermittent anticonvulsive therapy is recommended for children with one or more simple febrile seizures.” (AAP recommendations)
- antipyresis
21
Q
febrile seizures: recurrence risk
A
- recurrence risk
- young age at onset
- history of febrile seizure in a first-degree relative
- low degree of fever while in emergency department
- brief duration between the onset of fever and the initial seizure
- all 4 factors – recurrence risk is 70%
- 0 of 4 factors – recurrence risk is <20%
- Risk of epilepsy:
- Simple Febrile Seizures: 1-2%
- Complex Febrile Seizures: 5-10%
- risk of recurrent febrile seizures is 10%
- higher in children with first febrile seizure under age 1 year
22
Q
infantile spasms (west syndrome)
A
- term used to describe seizures that have characteristic clinical findings in age group 4 months to 8 months
- sudden adduction and flexion of limbs
- sudden adduction and flexion of the head and trunk
- usually occurs in clusters when patient is irritable or fatigued
- categories
- cryptogenic (40%)
- no etiology evident, normal development before seizures
- symptomatic (60%)
- association with perinatal and prenatal event or other identifiable cause
- have poor responses to anticonvulsants and poor intellectual prognosis
- cryptogenic (40%)
- EEG
- waking state EEG reveals chaotic high-voltage slow waves, random spikes & background disorganization
- treatment
- anticonvulsant therapy
- ACTH may be helpful
- ketogenic diet
23
Q
neurocutanous disorders
A
- Neurofibromatosis
- Tuberous Sclerosis
- Sturge-Weber syndrome
- disorders of tissue arising from neuroectoderm
- skin findings
- brain, spinal cord, eye manifestations
- hamartomas
- normal tissue growing at abnormal sites or abnormally rapidly
24
Q
neurofibromatosis
A
- two types of neurofibromatosis
- NF 1 and NF 2
- NF-1 epidemiology
- 50% of cases due to new mutations in the NF1 gene
- 40% of patients will develop medical complications of the disorder
25
neurofibromatosis type 1
* NF-1 is more prevalent than NF-2
* clinical criteria
* six or more café au lait spots
* axillary or inguinal freckling
* two or more Lisch nodules
* two or more neurofibromas or one plexiform neurofibroma
* distinctive osseous lesion
* optic glioma
* **first degree relative with NF-1**
26
cafe au lait spots
* present in nearly all neurofibromatosis patients
* predilection for trunk and extremities
* spares the face
* common normal finding but in patients with increased number of spots and larger size characteristic of NF-1
27
neurofibroma
* small, rubbery, usually involve the skin
* may be found viscerally and along peripheral nerves
* often appear in adolescence
* plexiform neurofibroma
* diffuse thickening of nerve trunk
* may produce overgrowth of extremity
28
complications of neurofibromas
* scoliosis
* learning disabilities
* hypertension (renal artery stenosis)
* disorders of the hypothalmus
* tumors
* brain
* eye (may manifest with strabismus)
29
treatment of neurofibromatosis
* treatment
* management of complications
* referral to a neurofibromatosis center
* genetic counseling
* prenatal testing is available if parent of possibly affected patient has known mutation
* brain MRI if any hint of optic glioma
* renal ultrasound with Doppler flow
* school assessment (individualized education plan)
30
neurofibromatosis 2
* prevalence of about 1 in 40,000
* criteria
* unilateral eighth nerve mass, particularly bilateral acoustic neuroma
* parent, sibling or child with NF-2 with either
* unilateral eight nerve mass or
* any two of the following
* neurofibroma
* meningioma
* glioma
* Schwannoma
* juvenile posterior subcapsular lenticular opacities
31
tuberous sclerosis
* autosomal dominant disorder characterized by proliferation of tubers from abnormal production of protein hamartin and tuberin
* prevalence of about 1 in 6,000
* clinical manifestations
* may present with seizures **(infantile spasms)**
* calcified tubers in the periventricular areas
* mental retardation
* hypopigmented skin lesions
* ash leaf macules
* shagreen patch
* adenoma sebaceum
* ash leaf spot
* hypopigmented macule or patch
* Wood’s lamp highlights appearance
* shagreen patch
* roughened, raised lesion with an orange peel, leathery texture
* often located in lumbosacral region
* sebaceous adenomas
* tiny, red, hyperpigmented nodules over the nose and cheeks
* subungual fibroma
* periungual fibroma
* commonly appear during adolescence
* treatment
* seizure control
* screening for complications
* renal ultrasound
* echocardiogram to look for rhabdomyomas
* ophthalmologist referral to look for hamartomas
* genetic counseling
32
Sturge-Weber disease
* etiology
* anomalous development of the vascular bed during early cerebral vascularization
* overlying leptomeninges richly vascularized and the brain beneath becomes atrophic and calcified
* clinical manifestations
* facial nevus (port-wine stain)
* seizures
* mental retardation
* intracranial calcifications
* hemiparesis
* port wine stain
* facial nevus involving upper face, eyelid
* glaucoma of the ipsilateral eye common complication
* radiologic diagnosis
* intracranial calcifications on CT scan
* unilateral cortical atrophy and ipsilateral dilatation of the lateral ventricle
* CT scan findings
* cerebral atrophy
* intracranial calcifications
* treatment
* conservative management if seizures and development problems mild
* moderate to severe disease
* recalcitrant seizures – lobectomy
* controversial decision
* risk of glaucoma - ophthalmologist intraocular pressure monitoring
* port wine stain - laser therapy
* developmental delay – special education
33
Guillain-barre syndrome
* acute inflammatory demyelinating polyradiculoneuropathy
* Different subtypes exist including axonal GBS, Miller Fischer variant
* disorder is thought to result from a post-infectious immune mediated process that predominantly affects motor nerves
* pathophysiology
* most likely grouping of disorders with peripheral nerve demyelination as a common mechanism
* occurs after infection in two-thirds of cases
* *Campylobacter* sp.
* CMV, EBV
* *Haemophilus influenzae*
* *Mycoplasma pneumoniae*
* viruses
* clinical manifestations
* fine paresthesias & tingling of toes and fingertips
* pain may be a prominent feature
* ascending weakness/paralysis
* lower extremity symmetric weakness
* ascends to arms and cranial nerves
* exam
* **diminished or absent deep tendon reflexes**
* **weakness**
* paralytic ileus
* poor respiratory effort
* bladder dysfunction
* autonomic dysregulation
* hypertension
* hypotension
* tachycardia/bradycardia
* abnormal sweating
* diagnosis
* MRI showing nerve root enhancement frequently seen
* electrophysiologic studies
* Specific autoantibodies can be sent
* CSF
* elevated protein
* Treatment
* **IVIG**
* plasmapheresis
* physical therapy
* watch for complications
* respiratory failure
* nutrition
* autonomic disturbance
34
transverse myelitis
* inflammation of the spinal cord
* thought to have a immunologic basis
* pathogenesis (proposed)
* cell-mediated immune response
* direct invasion of the spinal cord
* autoimmune vasculitis
* clinical manifestations
* back pain at level of lesion
* abrupt onset of progressive weakness; legs weak and flaccid
* areflexia
* sensory disturbances in the lower extremities below level of spinal lesion
* history of preceding viral infection
* sphincter disturbances
* laboratory findings
* lumbar puncture - non-specific
* increased protein
* pleocytosis with lymphocytes
* MRI (often diagnostic)
* localized edema of area of spinal cord
* treatment
* medications (controversial benefit)
* corticosteroids
* IVIG
* plasmapheresis
* supportive care
35
infant botulism
* acute, flaccid paralytic illness caused by the neurotoxin produced by soil organism *Clostridium botulinum*
* Gram-positive spore forming anaerobe
* found in fresh and cooked agricultural products
* HONEY - no honey under age 1
* botulinum toxin is heat labile
* forms of botulism
* wound
* food-borne
* infantile
* 95% of cases are between 3 weeks and 6 months of age
* almost half of cases are from California
* probably due to soil mixture
* history of parent working in/with soil - construction, landscaping etc
* botulinum toxin is carried by the bloodstream to peripheral cholinergic synapses
* binds irreversibly, blocking acetylcholine release and causing impaired neuromuscular and autonomic transmission
* loss of motor endplate function
* recovery requires re-growth of terminal unmyelinated motor neurons
* symmetric, descending paralysis
* cranial nerve palsies
* poor feeding
* weak suck
* feeble cry
* ptosis
* obstructive apnea
* laboratory confirmation
* detection of botulinum toxin in serum
* detection of *Clostridium botulinum* in feces
* CSF normal
* EMG defect in neuromuscular transmission
* treatment
* supportive care
* tube enteral feeding
* respiratory support
* positioning to avoid aspiration
* mechanical ventilation
* botulism immune globulin (BIG)
* reduces duration of illness if given early
36
tics
* quick, repetitive, irregular, briefly suppressible movements
* facial
* blinking
* grimaces
* twitches
* trunk and extremities
* tics occur in 20% or more of school children
* tics that are chronic occur in 1% of mainstream children
* usually last 1 month to 1 year
* stress/anxiety tend to increase the amount of tics
* treatment
* teach parents natural history
* disregard
* monitor for co-morbidities
* school problems
* attention deficit hyperactivity disorder
* obsessive-compulsive disorder
* sleep difficulties
37
Tourette's syndrome
* diagnostic criteria
* tics occur many times a day (usually in bouts) nearly every day or intermittently throughout a period of more than one year
* during this period there was never a tic-free period of more than three consecutive months.
* both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently
* onset before 18 years of age
* disturbance is not due to the direct physiological effects of a substance or a general medical condition (e.g. Huntington’s disease or post-viral encephalitis)
* treatment
* non-pharmacologic
* counseling
* adjustment of school and family members
* medication
* haloperidol
* fluoxetine
* clonidine
38
Spinal muscular atrophy types I, II, III
* group of disorders characterized by degeneration of motor neurons with compensation from re-innervation of an adjacent motor unit
* upper motor neurons are not involved
39
SMA type I, Wednig-Hoffman disease
* clinical manifestations
* severe hypotonia
* generalized weakness
* thin muscle mass
* involvement of the tongue (fasciculations)
* preservation of extraocular muscles
* laboratory diagnosis
* serum creatine kinase may be mildly elevated
* different from muscular dystrophy
* muscle biopsy - areas of atrophy, adjacent areas of hypertrophy
* molecular genetic diagnosis
* treatment
* no treatment to delay progression
* functional aids in type II or type III
* supportive care
* feeding NG feeds
* respiratory support
* oxygen
* pulmonary toilet
40
derangements of cranial size
* Head size derangements
* microcephaly
* head circumference more than 2 standard deviations below the mean for age
* macrocephaly
* head circumference more than 2 standard deviations above the mean for age
41
microcephaly
* chromosomal/genetic/metabolic disorder
* congenital malformation
* placental insufficiency
* perinatal hypoxia/trauma
* toxins (fetal alcohol syndrome, medications, maternal phenylketonuria)
* infections (TORCH)
* radiation
* familial
42
macrocephaly
* differential diagnosis
* benign familial macrocephaly
* increased intracranial pressure
* with dilated ventricles - hydrocephalus
* with mass effect - tumor, arachnoid cyst, porencephalic cyst
* megalencephaly (large brain)
* neurocutaneous disorder
* gigantism
* metabolic disorder
* lysosomal storage disease
* leukodystrophy – progressive white matter degeneration
* thickened skull
