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Flashcards in Neurology Deck (62):
1

Infantile spasms (West Syndrome) def=
What are the criteria?

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

2

Infantile spasms (categories and what lab reveals what)?

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
EEG
waking state EEG reveals chaotic high-voltage slow waves, random spikes & background disorganization

3

Infantile spasms (treatment)

treatment
anticonvulsant therapy
ACTH may be helpful
ketogenic diet(high fat diet so the body breaks fatty acids down to ketones and this is fuel for the brain which reduces seizures)

4

Guillain-Barre Syndrome (paralysis, what is it?)

acute idiopathic acquired inflammatory demyelinating polyradiculoneuropathy (peripheral nerves are affected)
disorder is thought to result from a post-infectious immune mediated process that predominantly affects motor nerves

5

Guillain-Barre Syndrome (who does it affect)

epidemiology
males > females
occurs in all ages but rarely in ages less than two years

6

Guillain-Barre Syndrome (clinical manifestations)

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

7

Guillain-Barre Syndrome (PE eval)

exam
diminished or absent deep tendon reflexes
paralytic ileus
poor respiratory effort
bladder dysfunction
autonomic dysregulation
hypertension
hypotension
tachycardia/bradycardia
abnormal sweating

8

Guillain-Barre Syndrome (dx)

diagnosis
electrophysiologic studies
mildly elevated ESR
CSF
elevated protein

9

Guillain-Barre Syndrome (treatment
)

treatment
IVIG
plasmapheresis
physical therapy
watch for complications
respiratory failure
nutrition
autonomic disturbance

10

Transverse myelitis (what is it and patho)

inflammation of the spinal cord with perivascular cuffing by lymphocytes
thought to have a immunologic basis
pathogenesis (proposed)
cell-mediated immune response
direct invasion of the spinal cord
autoimmune vasculitis

11

Transverse myelitis (clinical manifestations)

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

12

Transverse myelitis (labs findings)

laboratory findings
lumbar puncture - non-specific
increased protein
pleocytosis with lymphocytes
MRI (often diagnostic)
dwelling of area of spinal cord
EMG
normal early in disease
may show denervation at level of lesion

13

Transverse myelitis (treatment)

treatment
medications (controversial benefit)
corticosteroids
IVIG
plasmapheresis
supportive care
physical therapy

14

Infant botulism (what is it, who does it affect)

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
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

15

Botulism pathogenesis

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

16

Infantile botulism (PE)

symmetric, descending paralysis
cranial nerve palsies
poor feeding
weak suck
feeble cry
ptosis
obstructive apnea

17

Infant botulism (labs)

laboratory confirmation
detection of botulinum toxin in serum
detection of Clostridium botulinum in feces
CSF normal
EMG defect in neuromuscular transmission

18

Infant botulism (treatment)

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

19

Tics

quick, repetitive, irregular, briefly suppressible movements
facial
blinking
grimaces
twitches
trunk and extremities
twisting
flinging
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

20

Tics (treatment)

treatment
teach parents natural history
disregard
monitor for co-morbidities
school problems
attention deficit hyperactivity disorder
obsessive-compulsive disorder
sleep difficulties

21

Tourette’s syndrome (what is it)

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 one year
during this period there was never a tic-free period of more than three consecutive months.

22

Tourette’s syndrome (treatment)

treatment
non-pharmacologic
counseling
adjustment of school and family members
medication
haloperidol
fluoxetine
clonidine

23

Spinal muscular atrophy types I, II, III (what is it)

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

24

SMA type I, Werdnig-Hoffman disease (clinical manifestations)

clinical manifestations
severe hypotonia
generalized weakness
thin muscle mass
involvement of the tongue (fasciculations)
preservation of extraocular muscles

25

SMA type I, Werdnig-Hoffman disease (labs)

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

26

SMA type I, Werdnig-Hoffman disease (treatment)

treatment
no treatment to delay progression
functional aids in type II or type III
supportive care
feeding NG feeds
respiratory support
oxygen
pulmonary toilet

27

Head size derangements (Brain growth drives head growth)

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

28

Head size derangements (work up or observation)

review history with DDx in mind
follow the head growth pattern
neurologic exam
abnormal development, neurologic abnormalities, or accelerating or decelerating head growth lead to early cranial imaging

29

paroxysmal torticollis

attacks of head tilt, sometimes with vertigo +/-vomiting
requires ruling out posterior fossa pathology

30

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

31

cyclic vomiting

protracted attacks of vomiting, 1-4 times/hour, up to 5 days
requires ruling out epilepsy, GI disorder, urea cycle disorder

32

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

33

confusional migraine

episodic disorientation or combativeness, sometimes followed by headache
requires ruling out drug abuse, epilepsy, CNS ischemia

34

Migraine therapies.

migraine acute episode tx

sleep
acute treatment
acetaminophen, ibuprofen
sumatriptan, other triptans
rescue treatment
NSAIDs
promethazine
metoclopramide

35

Migraine prophylaxis

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

36

Headache: Summary

acutely rule out underlying pathology
address exacerbating factors
management plan
medications (start with acetaminophen, ibuprofen)
abortive agents
daily prophylaxis (neurologist)

37

Pseudotumor cerebri

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

38

Pseudotumor Cerebri
symptoms and signs of intracranial hypertension

acute
headache
pulse synchronous tinnitus
pain behind the eye
pain with eye movements
transient visual obscurations
blurred vision or double vision
CN VI paresis(one eye lateral oblique wont turn
vomiting
macrocephaly
altered behavior

chronic
growth impairment
optic atrophy
visual field loss
total blindness

39

Pseudotumor cerebri treatment

treatment of underlying causes
--weight loss is mainstay of therapy!!!!
treat anemia
medical treatment
-diuretics
-acetazolamide (Diamox) causes peripheral paresthesias - ----- and metallic taste to carbonated soft drinks
-furosemide (Lasix)
-glucocorticoids
-lumbar puncture=final tx
surgical treatment
optic nerve sheath decompression
lumboperitoneal shunt

40

Treatment of Idiopathic Intracranial HTN

Perimetry
NO VISUAL LOSS VISUAL LOSS
1. wt loss wt loss
2. sx HA tx Acetazolamide,
furosemide,
steroids

41

Seizures def.

epilepsy def.

a sudden, transient disturbance of brain function, manifested by involuntary, motor,
sensory, autonomic, or psychic phenomena
epilepsy= repeated seizures without evident cause
recurrent, unprovoked seizures

42

Potential causes of Seizure's?

infection (meningitis/encephalitis)
trauma
metabolic (hypoglycemia, hyponatremia)
hypoxic
tumor
malformation (hydrocephalus)

43

Nonepileptic paroxysmal events

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

44

EEG? as a lab for dx?

EEG
not sensitive and not specific
may help classify seizure type to determine therapy
may reveal subclinical seizures

45

Seizure tx

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)
(meds) Benzo's, long term anticonvulsant's

46

Febrile seizure.
What is it?
when should it concern you?

convulsion associated with a temp > 38.0° Celsius
child 6 months to 5 years of age
sudden onset and goes away
seizure in a younger infant should concern you
some children under 5 who develop febrile seizures may have persistent febrile seizures further on into childhood
history of previous a febrile seizures?
The high temp is not causing the seizure.....the thing causing the temp to go up is also causing the seizure....

47

simple febrile seizure – meets all criteria

complex febrile seizure – meets one or more criteria:

duration under 15 minutes (sent home)
total duration less than 30 minutes if in series
no recurrence in 24 hours
no focal features
most commonly genralized and tonic clonic.

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

48

febrile seizure is not to ?

central nervous system infection or inflammation
no acute systemic metabolic abnormality that may produce convulsions

49

Febrile seizure etiology?

infection
human herpes virus 6 (roseola)
influenza
rotavirus

immunization-related fever

predisposing factors
heredity
inhibitory neurotransmitter production
maternal alcohol intake and smoking during pregnancy raises risk 2-fold
Oten the 1st sign of illness

50

Fevers in infants & children under 3 months of age

10% who appear well have a temp over 38°C have a serious bacterial infection or meningitis

51

Fevers in infants & children 3-36 months of age

2% who have a temperature above 39° C are found to have bacteremia

52

AAP recommends strong 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

53

Febrile seizure tx

ABCs
anticonvulsants
if seizure is prolonged (>10 minutes)
watch for respiratory compromise
drugs in office or ED
benzodiazepine (midazolam, lorazepam)
fosphenytoin
rectal diazepam

54

Role of preventative seizures

antipyresis
around-the-clock use not shown to be of benefit to all children with febrile seizures
intermittent use warranted
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)
continuous phenobarbital or valproate reduce the risk of recurrent febrile seizures but have significant side effects

55

recurrence risk febrile seizures

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%

56

Risk of Epilepsy

risk of epilepsy is 2% (twice as high as general population)
family history of febrile seizures, ethnicity, gender are not risk factors for development of epilepsy in those with febrile seizures

57

Neurocutaneous disorders

disorders of tissue arising from neuroectoderm
skin findings
brain, spinal cord, eye manifestations
hamartomas
normal tissue growing at abnormal sites or abnormally rapidly

58

Neurofibromatosis;
2 types

two types of neurofibromatosis
neurofibromatosis type 1
neurofibromatosis type 2
NF-1 epidemiology
prevalence 1 in 4000
50% of cases due to new mutations in the NF1 gene
40% of patients will develop medical complications of the disorder

59

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
(café au lait spots
present in nearly all neurofibromatosis patients
predilection for trunk and extremities
spares the face)

60

Neurofibromatosis;
neurofibroma

neurofibroma
small, rubbery, usually involve the skin
may be found viscerally and along peripheral nerves

61

Neurofibromatosis tx?

management of complications
referral to a neurofibromatosis center
genetic counseling

62

Neurofibromatosis type 2

NF-2

criteria
unilateral eighth nerve mass, particularly bilateral acoustic neuroma