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Pediatrics > II. Neurology > Flashcards

II. Neurology Flashcards

1
Q

Most severe form of neural tube defect (dysraphism) due to failure of the neural tube to close spontaneously between the 3rd to 4th week of in utero development with the ff signs: flaccid paralysis of the LE, absence of DTRs, lack of response to pain and touch, hip subluxation, clubfeet, bowel and bladder incontinence, associated with hydrocephalus (type II Chiari)

A

Meningocoele (Tx: Folic acid 0.4mg once a day)

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2
Q

Due to failure of closure of the rostral neuropore resulting to a large calvarium, meninges, and scalp associated with a rudimentary brain consisting of connective tissue, vessels, and neuroglia

A

Anencephaly

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3
Q

Absent cerebral convolutions and poorly formed sylvian fissure due to faulty neuroblast migration

A

Lissencephaly (agyria)

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4
Q

unilateral or bilateral clefts within the cerebral hemispheres

A

Schizencephaly

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5
Q

Presence of cysts or cavities within the brain in the sylvian fissure

A

Porencephaly

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6
Q

Due to defective cleavage of prosencephalon and inadequate induction of the forebrain structures with evidence of noncleaved midline brain structures

A

Holoprosencephaly

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7
Q

A condition with the ff signs: setting sun sign (eyes deviate downward due to impingement of dilatged suprapineal recess on the tectum), long-tract signs (brisk DTRs, spasticity, clonus, babinski sign), cracked pot sensation on percussion of the skull and Macewen sign (separation of sutures)

A

Hydrocephalus (Tx: Acetazolamide and Furosemide provde temporary relief by reducing CSF production)

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8
Q

Type of hydrocephalus due to abnormality of the aqueduct (aqueductal stenosis) or a lesion on the 4th ventricle resulting to obstruction within the ventricular system

A

Obstructing or Non-communicating type

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9
Q

Type of hydrocephalus due to subarqachnoid hemorrhage resulting to obliteration of the subarachnoid cisterns and malfunction of the arachnoid villi

A

Non-obstructing or communicating type

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10
Q

Major cause of VP shunt complications

A

Staph. epidermidis

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11
Q

Sudden cessation of motor activity or speech with a blank facial expression and flickering of the eyelids (never associated with an aura or postictal state, rarely persist longer than 30secs and patients do not lose body tone)

A

Absence seizures (Tx: Eva - Ethosuximide and Valproic Acid)

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12
Q

Rhythmic clonic contractions alternating with relaxation of all muscle groups with a post ictal state (vomiting and intense headache), loss of sphincter control, associated with an aura, sudden loss of consciousness, cyanosis, and apnea

A

Generalized Tonic-Clonic seizure (Tx: P-P-Ca-La-Va-G: phenobarbital, phenytoin, carbamazepine, lamotrigine, valproic acd, gabapentin)

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13
Q

Repetitive seizures consisting of brief often symmetric muscular contractions with loss of body tone and falling or slumping forward

A

Myoclonic seizures

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14
Q

Begin at 4-8 months, brief symmetric contractions of the neck, trunk, extremities, and EEG shows hypsarrythmia (high voltage bilaterally asynchronous, slow wave activity)

A

Infantile Spasms (Tx: Vigabatrin)

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15
Q

Most common seizure disorder in chooldhood, peaking at 14-18 months, rare before 9 months and after 5 yrs old, normal neurologic exam, normal EEG, with a positive family history

A

Febrile seizure

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16
Q

Lasts a few secs and rarely greater than 15mins, occurs only once in 24hrs

A

Simple febrile seizure

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17
Q

Lasts greater than 15mins, repeated convulsions occuring with 24hrs, with focal seizure activity

A

Complex febrile seizure

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18
Q

One seizure lasting 30mins or multiple seizures during 30mins without regaining consciousness usually caused by breakthrough seizures (missed doses of anti-epileptic drugs)

A

Status Epilepticus (Tx: Phenytoin)

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19
Q

Recurrent headache with symptom free intervals and at least 3 of the ff: (+) family history, relief following sleep, unilateral location, assoc aura, abdominal pain, nausea and vomiting, throbbing in character (F-R-U-A-N-T)

A

Migraine (Tx: 1. Analgesics - acetaminophen or Ibuprofen 2. Antiemetics - metoclopramide 3. Prophylaxis - Propanolol or Flunarizine)

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20
Q

A phenomenon associated with high CNS hydrogen and potassium ions with release of ntrous oxide that leads to excitation of trigeminal vascular system, release of VIP, vasodilation, extravasation of plasma proteins from dural vessels, inflammation of dural vessels and excitation of pain sensitive receptors

A

Cortical spreading depression

21
Q

Most prevalent type of migraine, family history in the maternal side with throbbing or pounding pain in bifrontal and temporal areas, light neadedness, photophobia, osmophobia, and phonophobia

A

Migraine without aura

22
Q

Type of migraine with blurred vision, photopsia, and fortification spectra (brilliant white zigzag lines)

A

Migraine with aura

23
Q

Treatment for status migrainosus (headache lasting greater than 3 days)

A

IV Prochlorperazine

24
Q

Common after onset of puberty, band-like tightness or pressure around the head usually on the frontal region, sometimes in the occipital region, and not associated with nausea and vomiting

A

Tension Headache

25
Q

A condition associated with mulberry tumors, rhabdomyosarcoma of the heart, bilateral angiomyolipomas, ashleaf lesions in the abdomen, and subungual or periungual fibromas

A

Tuberous sclerosis

26
Q

Tuberous sclerosis complex gene 1 encodes for what protein?

A

Hamartin

27
Q

Tuberous sclerosis complex gene 2 encodes for what protein?

A

Tuberin

28
Q

Typically present in the subependymal region, undergo calcification and project into the ventricular cavityproducing a candle dripping appearance characteristic of tuberous sclerosis

A

Tubers

29
Q

A roughened raised lesion with an orange-peel consistency located primarily in the lumbodacral region characteristic of Tuberous sclerosis

A

Shagreen patch

30
Q

Result of an abnormality of neural crest dfferentiation and migration during the early stages of embryogenesis located on chromosome 17

A

Neurofibromatosis (Von Recklinghausen Disease)

31
Q

Most prevalent NF characterized by cafe au lait macules, axillary or inguinal freckling, iris Lisch nodules, neurofibromas, optic glioma

A

NF-1

32
Q

Type of NF wiht bilateral 8th nerve masses (acoustic neuroma), neurofibroma, meningioma, glioma, schwannoma

A

NF-2

33
Q

Most common cause of meningitis in the 1st 2 months of life

A

Group B streptococcus, E coli, Listeria monocytogenes

34
Q

Most common cause of meningitis in 2 months to 12 years old

A

Strep pneumoniae (Tx: 3rd gen cepha or penicillin IV for 10-14 days), H. influenzae, N. meningitidis (Tx: Penicillin IV for 5-7days)

35
Q

CSF findings in bacterial meningitis

A

Pleocytosis, high protein, and low glucose

36
Q

Most common causes of viral meningitis

A

Echovirus, coxsackie virus, adenovirus, CMV, HSV

37
Q

CSF findings in viral meningitis

A

normal glucose, normal to slightly increase increased proteins,lymphocytosis

38
Q

Results from embolization due to CHD with R to L shunts, meningits, chronic OM and mastoiditis, face and scalp infections, orbital cellulitis, dental infections, penetrating head injuries, VP shunt infections

A

Brain Abscess (Cerebrum 80% of the time, Tx: 1. unknown cause: 3rd gen cepha + metronidazole 3. Head trauma: Nafcillin or Vancomycin with 3rd gen + metro 3. CHD: penicillin + metro 4. Infected VP shunt: vanco + ceftazidime 3. immunocompromised: broad spectrum + amphotericin, duration: 4-6 weeks)

39
Q

Organisms implicated in brain abscess

A

S. aureus, streptococci, anaerobes, gram neg aerobes (Proteus, pseudomonas, hemophilus, citrobacter)

40
Q

A condition caused by a non-responsive motor end plate to normal release of the synaptic cleft due to circulating receptor binding Abs with the ff signs and symptoms: ptosis, dysphagia, facial ms weakness, ms fatigue, proxmal ms weakness diminished tendon reflexes and fasciculations, myalgiasm and sensory deficits more symptomatic during the day

A

Myasthenia gravis

41
Q

Diagnostic test for MG

A

Tensilon test using Edrophonium chloride

42
Q

Treatment for MG

A

Neostigmine, thymectomy, plasmapharesis

43
Q

Acute unilateral facial nerve palsy not associated with other cranial neuropathies preceded by a viral infection (EBV, HSV, mumps) 2 weeks earlier

A

Bell’s Palsy

44
Q

Post infectious polyneuropathy affecting the motor system resulting in LE extremity weakness and progressively involves the trunk, upper limbs, and bulbar ms

A

GBS/Acute demyelinating polyradiculoneuropathy (Tx: IVIg for 5 days, high dose pulse methylprednisolone IV for relapses)

45
Q

A condition associated with GBS characterized by acute ophthalmoplegia, ataxia, and areflexia

A

Miller-Fischer syndrome

46
Q

CSF finding diagnostic of GBS

A

Dissociation between high CSF protein and a lack of cellular response (albuminocytologic dissociation)

47
Q

Medulloblastomas are commonly found in the cerebellum with a histological finding of circular patterns of tumor cells surrounding a center of neutrophils called?

A

Homer-Wright rossettes

48
Q

Most common infrratentorial tumor with the best pronosis

A

Cerebellar astrocytoma

49
Q

A tumor with solid and cystic areas that tend to calcify, exerting pressure in the optic chiasm producing bitemporal visual field deficits. Patients with this condition have short stature

A

Craniopharyngioma

Pediatrics (7 decks)

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