neuroped Flashcards
1
Q
most severe form of NTD
A
menigocele(dysraphism)
2
Q
when does the neural tube close spontaneously
A
bet 3rd and 4th wk AOG
3
Q
underlying causes of NTD
A
radiation; drugs; malnutrition; genetic determinants
4
Q
how to prevent myelomeningocele
A
maternal periconceptual use of folic acid reduces its incidence by 50%(started before conception until at least 12wks AOG); 0.4mg/day
5
Q
location of majority of NTD
A
lumbosacral (75%)
6
Q
manifestations of NTD
A
flaccid paralysis of LE; absent DTR’s; lack of response to pain and touch; hip subluxation; clubfeet; bowel and bladder incontinence; associated with type II chiari hydrocephalus
7
Q
cause of anencephaly
A
failure of closure of the rostral neuropore
8
Q
absent cerebral convolutions; poorly developed sylvian fissure; failure to thrive; mircrocephaly; developmental delay; seizures; faulty neuroblast migration
A
lissencephaly (agyria)
9
Q
uni or bilateral clefts within the cerebral hemispheres; severe MR; seizures; microcephaly; spastic quadriparesis
A
Schizencephaly
10
Q
presence of cysts or cavities within the brain
A
porencephaly
11
Q
due to defective cleavage of prosencephalon and inadequate induction of the forebrain structures; evidence of noncleaved midline brain structures
A
holoprosencephaly
12
Q
type of hydrocephalus where obstruction is within the ventricular system; abnormality of the aqueduct or a lesion in the 4th ventricle
A
obstructive or noncommunicating type
13
Q
type of hydrocephalus where there is obliteration of the subarachnoid cisterns and malfunction of the arachnoid villi; follows a subarachnoid hemorrhage; leukemic infiltrates
A
nonobstructive or communicating type
14
Q
site of CSF production
A
choroid plexus epithelium within the cerebral ventricles
15
Q
how is csf reabsorbed
A
arachnoid villus cells; which are located in the superior sagittal sinus return CSF to the blood stream within the vacuoles(pinocytosis)
16
Q
eyes may deviate downward due to impingement of the dilated suprapineal recess on the tectum
A
setting-sun sign
17
Q
separation of sutures
A
macewen sign
18
Q
foreshortened occiput
A
chiari malformation
19
Q
prominent occiput
A
dandy-walker malformation
20
Q
major cause of complications of shunting in hydrocephalus
A
Staph epidermidis infxn
21
Q
present when 2 or more unprovoke seizures occur at an interval greater than 24hrs apart
A
epilepsy
22
Q
consciousness retained or not in simple partial seizure
A
retained
23
Q
consciousness retained or not in complex partial seizure
A
impaired
24
Q
type of seizure where there is sudden cessation of motor activity or speech with a blank facial expression and flickering of the eyelids
A
absence seizure
25
definition of absence seizure
never associated with an aura; rare in <5yo; not associated with postictal state; rarely persist longer than 30s; no loss of body tone
26
seizure associated with an aura; sudden loss of consciousness and patient may become cyanotic and apneic; there is loss of sphincteric control
generalized tonic-clonic
27
repititive seizures consisting of brief often symmetric muscular contraction with loss of body tone and falling or slumping forward
myoclonic seizures
28
Tx for GTC seizure
PPCaLaVaG; Phenobarbital; Phenytoin; Carbamazepine; LAMOTRIGINE; Valproic Acid; Gabapentin
29
Tx for absence seizure
Ethosuximide; Valproic acid
30
Tx for infantile spasms
Vigabatrin
31
purpose of ketogenic diet in seizures
anticonvulsant effect due to increased beta hydroxybutyrate and acetoacetate resulting from ketosis
32
most common seizure disorder in childhood
febrile seizure
33
characteristics of febrile seizure
rare before 9mos and after 5yrs old; peaks at 14-18mos; normal EEG; (+) family history
34
febrile seizure with duration >15mins; repeated convulsions occur within 24hrs; focal seizure activity
complex febrile seizure
35
one seizure lasting 30mins or multiple seizures during 30mins without regaining consciousness
status epilepticus
36
usual cause of status epilepticus
breakthrough seizures; missed doses of anti epileptic drug
37
DOC for status epilepticus
diazepam and phenytoin
38
recurrent headache with symptom-free intervals and at least 3 of the following 1Family history 2Relief ff sleep 3Unilateral location 4Associated aura 5Abdominal pain 6Nausea and Vomiting 7Throbbing in character
migraine
39
most prevalent type of migraine; throbing or pounding; bifrontal or temporal areas; intense nausea and vomiting; (+) family hx in 90% usually maternal
migraine without aura
40
migraine with sensory symptoms; fortification spectra (brillian white zigzag lines); Alice in Wonderland syndrome
migraine with aura
41
migraine that has unilateral sensory or motor signs that may persist for days; good prognosis in older child or adolescent
hemiplegic aura
42
migraine associated with vasoconstriction of basilar and posterior cerebral arteries; girl<4yo at risk
basilar-type migraine
43
mid-abdominal pain with pain-free periods between attacks; at least 2 of the ff symptoms: anorexia; nausea; vomiting; pallor; pain may persist from 1-72hrs
abdominal migraine
44
persistent headache lasting >3days
status migrainosus
45
Tx for status migrainosus
Prochorperazine IV 0.15 mkd max 10mg
46
Prophylactic treatment for migraine (more than 2-4 severe episodes monthly)
propranolol 10-20mg TID for >7yo; Flunarizine 5mg at bedtime
47
TSC1 encodes for what protein
hamartin
48
TSC2 encodes for what protein
tuberin
49
candle-dripping appearance in the subependymal region
tuberous sclerosis
50
heterogeneous dse with wide clinical spectrum varying from severe MR and incapacitating seizures to normal intelligence and a lack of seizures; often within the same family
tuberous sclerosis
51
roughened; raised lesion with an orange-peel consistency primarily in the lumbosacral region in tuberous sclerosis
Shagreen patch
52
manifestations of tuberous sclerosis
infantile spasms; hypopigmented ASH leaf lesions; calcified tubers in the periventricular areas; subungual/periungual fibroma;mulberry tumors; rhabdomyosarcoma of the heart; bilateral angiomyolipomas
53
Management of Tuberous Sclerosis
seizure control and baseline 2D echo; CXR; renal UTZ
54
most prevalent type of Neurofibromatosis
NF1
55
Dx of NF1
2 of the ff: 1. 6 or more cafe au lait macules>5mm in prepubertals and >15mm in postpubertal individuals with sparing of the face; 2. 2 or more Lisch nodules; 3. 2 or more neurofibromas or one plexiform neurofibroma; 4. distinctive osseus lesion; 5. Optic glioma; 6. 1st degree relative with NF1; 7. high incidence of learning disabilities; 8. majority occurs in the paternal germline
56
Dx of NF2
1 of the ff: 1. bilateral acoustic neuroma; 2. parent; sibling; or child with NF2 and either unilateral acoustic neuroma or any 2 of the ff: 1. neurofibroma; 2. meningioma; 3.glioma; 4. schwannoma
57
hamartomoas located within the iris
Lisch nodules
58
Management of NF2
genetic counseling and early detection of treatable conditions or complications
59
chromosomal location of NF gene
Chromosome 17
60
aka von Recklinghausen dse
Neurofibromatosis
61
most common cause of bacterial meningitis in 1st 2mos of life
GBS; gm(-) enteric bacilli; Listeria monocytogenes
62
most common cause of bacterial meningitis in 2-12mos of life
Strep pneumoniae; H. influenzae; N. meningitidis
63
mode of transmission of bacterial meningitis
hematogenous from a distant site of infection
64
why is there neck rigidity in meningitis
inflammation of spinal nerves and roots produce meningeal signs of irritation
65
complications of meningitis
hydrocephalus; subdural effusions; SIADH may exacerbate edema leading to hyponatremic seizures
66
contraindications to LP
evidence of increased ICP; severe cardiopulmonary compromise; infection of the skin overlying the site
67
Tx for meningitis due to N. meningitidis
IV Penicillin for 5-7 days
68
Tx for meningitis due to S. pneumoniae
3rd gen Cephalosporin or IV Penicillin for 10-14 days
69
rationale of giving dexamethasone IV in HiB meningitis
less fever; lower CSF protein; reduced auditory damage
70
CSF analysis in viral meningitis
normal glucose; normal or slightly eleveated protein; lymphocytosis
71
where is cerebral abscess commonly located
cerebrum (80%); majority are single
72
etiology of cerebral absces
embolization due to RL shunt; meningitis; chronic OM or mastoiditis; face and scalp infections; orbital cellulitis; dental infections; penetrating head injuries; VP shunt infections
73
Causative agent in brain abscess
S.aureus; Strep; anaerobes; gm(-) aerobic bacilli(Proteus; Pseudomonas; Haemophilus; Citrobacter)
74
Dx of cerebral abscess
(+) blood culture in 10%; CSF not done due to herniation; cranial CT and MRI (most reliable methodS)
75
management of cerebral abscess
empiric antibiotics depend on the probable pathogens; unknown cause-3rd gen Cephalosporin + Metronidazole
76
Management of cerebral abscess due to head trauma or neurosurgery
Nafcillin or Vancomycin with 3rd gen Cep + Metronidazole or Meropenem as monotherapy
77
Management of cerebral abscess due to CHD
Penicillin + Metronidazole
78
Management of cerebral abscess due to infected VP shunt
Vancomycin + Ceftazidime
79
Management of cerebral abscess due in immunocompromised px
broad spectrum + Amphotericin B
80
Indications for sugery in cerebral abscess
1(+) gas in the abscess; 2Multiloculated abscess; 3Posterior fossa location; 4Fungal Cause; 5Associated infections like mastoiditis; periorbital abscess; sinusitis
81
duration of antibiotics in the Tx of cerebral abscess
4-6wks
82
dse characterized by immune-mediated neuromuscular blockade whre postsynaptic muscle membrane or motor endplate is less responsive than normal in releasing Ach
Myasthenia Gravis
83
earliest and most constant signs in Myasthenia Gravis
ptosis and some degree of EOM weakness
84
manifestations in MG
dysphagia and facial weakness; poor head control due to weak neck flexors; weakness involves limb-girdle and distal muscle of the hands; (fasciculations; myalgias and sensory symptoms do not occur); diminished tendon stretch reflexes
85
characteristic feature of MG
fatigue of muscles
86
what is more affected muscles in MG
proximal rather than distal muscle weakness
87
test where ptosis and ophtalmoplegia improve within a few seconds after administration of Edrophonium chloride IV in MG
Tensilon test
88
Dx of MG
EMG is more spefically diagnostic thatn muscle biopsy (decremental response is seen as a result of repititive nerve stimulation
89
Tx of MG
Neostigmine 0.4mg/kg IM q4-6hrs; Atropine to treat cholinergic crisis; Prednisone; Thymectomy when there is high titers of anti-Ach receptors and who are symptomatic for <2yrs; plasmapharesis to steroid nonresponders
90
acute unilateral facial nerve palsy not associated with other cranial neuropathies; usually develops abruptly about 2wks after a systemic viral infxn
Bell palsy
91
disease characterized by an autoimmune exn that develops in response to a previous infection that leads to aberrant demyelination of peripheral nerves and ventral motor nerve roots
Guillain-Barre syndrome aka demyelinating polyradiculoneuropathy
92
syndrome associated with Guillain-Barre that presents as acute opthalmoplegia; ataxia; and areflexia
Miller-Fisher syndrome
93
type of paralysis characterized by weakening beginning in the lower extremities and progressively involves the trunk; upper limbs; and bulbar muscles seen in Guillain-Barre
Landry ascending paralysis
94
clinical features predictive of poor outcoime with sequela
CN involvement; need for intubation; maximum disability at time of presentation
95
last function to recover in GBS
tendon reflexes and lower extremity weakness
96
Dx of GBS
albuminocytologic dissociation(high CSF protein with lack of cellular response); reduced motor NCV
97
Management of GBS
admit for observation bec ascending paralysis may occur within 24hrs; if rapidly progressive ascending paralysis: IVIG for 5 consecutive days; high dose pulse methylprednisolone IV for relapses
98
noprogressive disorder of posture and mov't often associated with epilepsy and abnormalities of speech; vision; and intellect resulting from a defect or lesion of the developing brain
Cerebral Palsy
99
clinical manifestations of spastic hemiplegia in CP
arms>legs; difficulty in hand manipulation obvious by 1yo; delayed wlaking or walks on tiptoes; spasticity apparent specially in the ankles; seizures and cognitive impairment
100
clinifcal manifestations in spastic diplega
bilateral spasticity of the leg; commando crawl; increased DTR's +Babinski; normal intellect
101
clinical manifestations in spastic quadriplegia
most severe form of CP due to marked motor impairment of all extremities and high association with MR and seizures; swallowing difficulties
102
2nd most commmon malignancy in childhood; most common solid tumors in childhood
brain tumors
103
2 distinct patterns of presentation of brain tumors
symptoms and signs of increased ICP and focal neurologic signs
104
signs in px with brain tumors that may indicate herniation of cerebellar tonsils
head tilting and nuchal rigidity
105
prominent signs in posterior fossa tumors
nystagmus and ataxia
106
more common location of brain tumors
infratentorial
107
age at which 2/3 of brain tumors occur
2-12yo
108
malignant brain tumor most often found in the cerebellum; M>F; heterogeneous enhancements in the cerebellum often invading the 4th ventricle and can cause obstructive hydrocephalus
Medulloblastoma
109
circular patterns of tumor cells surrounding a center of neutrophils found in Medulloblastoma
Homer-Wright rosettes
110
most common primary brain tumor; best prognosis; causes hydrocephalus; resection with 90% 5year survival rate
cerebellar astrocytoma
111
Tx of Medulloblastoma
surgery + irradiation; 80-90% 5year survival rate
112
brain tumor that is characterized by solid and cystic areas that tend to calcify; short stature; pressure to optic chiasm may cause bitemporal hemianopsia
craniopharyngioma
113
brain tumor that causes decreased visual acuity and pallor of the discs; 25% have NF; hyperalert and euphoric despite being emaciated; invasion of the hypothalamus leads to obesity or DI
optic nerve glioma
114
diagnostic imaging use to delineate brain tumors
cranial MRI
115
term used for implantation of radiation seeds for brain tumors
brachytherapy
