נוירולוגיה Flashcards

1
Q

What define simple fabrile seizure?

4 criteria

A
  • time < 15 min
  • 1 episode in 24h
  • generlazied sezuire
  • normal neurologic exam- w/o focal findings
  • no Tx needed
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2
Q

Tx for complex febrile siezure?

A

hospitalization + EEG, neurologic consulation and consider brain imgaing (mainly MRI)

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3
Q

Is focal seizure during fever is consider simple or complex febrile seizure?

A

Complex

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4
Q

Status Epilepticus

A

seizure > 30 min / w/o returning to concsious btwn episodes

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5
Q

סיכון להישנות וסיכון לאפילפסיה אחרי פרכוס חום פשוט

A
  1. אפילפסיה- 1% כמו באוכ’ הכללית
  2. סיכון להישנות - 30-50%
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6
Q

what are the 3 major risk factors for reccurent of febrile seizures?

A
  1. age < 1 yr
  2. less then 24h since febrile ilness
  3. low fever ~39
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7
Q

Which electrolyte abnormality is incorrelation with reccurency of fabrile siezures?

A

Hyponathermia during siezure

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8
Q

What is the most significant risk factor for future epilepsy?

A

neuro-development abnormality 33%

just think about autisim

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9
Q

Tx for kids with focal seizures?

A

Carbamazepine (tegretol)

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10
Q

Seizure with low conciussnes level + staring and automatism is most likely +++++++++ ______________ seziure

A

complex partial = פוקלי עם פגיעה בהכרה

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11
Q

Typical EEG finding of Absance ?

A

3-Hz spike-and-wave

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12
Q

Tx for absance ?

A

Etoxosumide

if tonic clonic- velporate

most will resolve in early adulthood

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13
Q

A parent come with a 5 year old bot who woke up at night with
ריור ותנועות ביד עם קושי בדיבור

מה האבחנה הכי סבירה? ומה הטיפול
ומה הפרונגנוזה

A

Roland epilapsy = simple focal

Tx- Carbamazepine
if few events- Tx not require

age group- 3-10
mainly resolve in adulthood

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14
Q

all of the following are triggers for which type of epilepsy?

alcohol
Sleep deprivation
Flickering light

A

Juvenile myoclonic epilepsy

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15
Q

Tx for Juvenile myoclonic seziures?

A

1st line- Valporic acid
if childberaing potentioal- Lamotrgins or levetiracem

lifelong therpy this condition tends not to resolve

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16
Q

EEG finding of Juvenile Myoclonic epilepsy?

A

polyspikes brought with photic stimulation

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17
Q

ילד בן 15 מתייצג עם תנועות ג’רקיות בבוקר וקלאמזיות .
ב-EEG תחת ריצודי אורות נצפו גלים בתדירות 4-6 הרץ

מהי האפילפסיה הסבירה?

A

Juvenile myoclonic epilepsy

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18
Q

A kid age 3 represent with neurodevelopment delay and 2 types of seizures.
on EEG theres a slow spike wave

what is the most likely underline cause?

A

Lennox gastu syndrome

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19
Q

which syndrome is a/w
* Infantile spasm
* hypsarrythmia
* nerodevelopment delay

A

West syndrome

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20
Q

if a baby (most under 1 yrs old) present with infantile spasm.
which test must be done in order to rule out which type of situation?

A

MRI
rule out Tuberous sclerosis

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21
Q

Tx for infantile spasm?

A

ACTH

unless its Tubreous sclerosis&raquo_space; Vigabatrin

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22
Q

איזו תסמונת מתייצגת עם התפתחות שפתית תקינה עד גיל 3-7 ואז רגרסיה ללא פגיעה ביכולת החברתיות

EEG- status epilepticus stractue in non-REM sleep

A

Landau Kleffner syndrome

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23
Q

Tx for Status epilepticus
first line + first recurrence
2nd line
3rd line

A
  • ** first line + first recurrence-** Benzodiasepins
  • 2nd line- phentoin / velporate / labitaracem
  • 3rd line- sedation and vantilation
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24
Q

Tx options for migrane ppx?

A
  1. CCB
  2. propronelol
  3. amitriplyine
  4. anti-epileptic medications
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25
Q

Cyclic vomiting syndrome is a/w with risk for developing which type of condition?

A

Migranse

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26
Q
  • A kid present with 8 cafe-lu eit lesions and frakels around axilla area.
  • on eye exmination the kids have 3 hemratomoas around the iris

which syndrome is the most possible cause?

A

NF 1
AD

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27
Q

What are clinical findings needed for Dgx of NF1

7 criteria

A

clinical 2 of the following
1. cafe aulei spots > 6
2. frakels in axilla and groin (80% until < 6 age)
3. Lisch nodules- > 2 in iris
4. Neurofibromas- > 2, or one flexiform type.
5. bone abnormality- thin cortex of the bones or sphenoidal dysplasia.
6. Optic gliomas
7. 1st dagree relative with dgx of NF1

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28
Q

Complications of NF1

A

Wilms tumor
HTN
Renal artery stnosis
pheochromocytoma

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29
Q

Most common cause of death in NF1 pt?

A

malignancy

תוחלת חיים 15 שנה פחות

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30
Q

a kid present with hypopigmentation lesions and apple like lesion in lumbosacral resions. which hear condition is most likley can be seen in this syndrome?

A

Rhabdomyomas

Tuberous sclerosis

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31
Q

Ash leaf lesions
Shagreen patch
epilepsy» infantile spasm
autism
rabhdomyomoas in heart

all of this finding a/w?

A

Tuberous sclerosis

31
Q

What is the classical finding in the brain tuberous sclerosis?

A

Cortical tubor

31
Q

Tx for tuberous sclerosis

A

Vigabatrin- anti-epileptic

32
Q

managment of tuberous sclerosis

A

Vigabatrin- seizures
MRI brain + kidney US every 1-3 yrs

33
Q

Port wine stains on face + train truck apperance on brain

are a/w which type of disease

A

Straug weber syndrome

34
Q

Which type of seziures are most a/w strauge weber

A

Tonic clonic seziure and the contra-lateral side of the port wine lesions

35
Q

Which eye complication is a/w Straug weber

A

Gleucoma due to abnormal vessels

36
Q

Which type of NTD is present with hypotonic paralysis of lower limbs, reccurent UTI, problems to control on peing

A

Myelomeningocele

Urgent surgery

37
Q

When in Essential tremor the tremor is worse? during activity or rest?

A

during activity

38
Q

Which syndrome is a/w normal development in girl until 6-18 months and then regression + aq. microchepaly , ASD, sterotype hands movment

A

RETT syndrome

39
Q

איזו מחלה אוטוזומלית רסציבית נמצאת באסוצאציה עם כיבים בקרנית
הקאות ציקליות, עקמת, חוסר ברפלקסים גידיים

A

**Familial dysautonomia
**

פוגע בעצבים פריפריים, מתאפיין בירידה במספר סיבי המיאלין האחראים על הולכת כאב, טמפ טעם ופונ’ אוטנומיות

40
Q

most common reasons for Cerebral palsy?

A

Intra-uterine Reasons 80%

10%- delivery asphixia

41
Q

Dgx of Cerebral palsy

A

clinical presenatation
+
Brain MRI

42
Q

most common presentation of CP?

A

דיפלגיה ספסטית - 35%

ספסטיות בשתי הרגליים יותר מאשר בידיים. רפלקסים פתולוגים, איחור מוטורי

פגות, איסכמיה, זיהומים, מטבולי

43
Q

common etiologies for spastic hemiplagia

A

intra-utrine damage
brain malformations

44
Q

ילד הפונה למרפאה נראה כי מטונוס שרירי גבוה רק בצד אחד של הגוף ויותר בידיים מאשר הרגליים.
הוא מרבה ללכת על קצות האצבעות ובבדיקת רפלקסיים נצפו רפלקסיים ערים בצד הפגוע.

מהי הסיבה האפשרית?

A

המיפלאגיה ספסטית

type of CP
נובע בעיקר מפגיעה תוך רחמית

45
Q

Which Ab are a/w ADEM (acute disseminated encephalomylitis )

A

anti-MOG

46
Q

A child present lethragy, fever, headaces, seizures, including status epilepticus. with neurological deficit.

his mother mentioned that month ago he had acute fever ilness.
on MRI there are multifocal lesions in white and gray metter.

what is the common cause?

A

ADEM

47
Q

Tx for ADEM

A

Steroids IV

refactory- IVIG or plasmapheresis

48
Q

Risk factor for Psuedotumor cerebrii

A

Obesity
Rtinoids, TCA
polycitemia
hypothyroidism

49
Q

כל הבאים הינם קליניקה טיפוסית של איזה מצב?

כאב ראש פרונטלי כרוני
הפרעות ראייה
דיפלופיה משנית לפגיעה בעצב 6
פפילדמה

בנוכחותת הדמייה תקינה ולחץ פתיחה מוגבר ללא שינויים

A

PTC
פסואודטומור סרבריי

50
Q

Complication of Psuedotumor cerebrii

A

atrophy of optic nerve&raquo_space; blindness

51
Q

Tx for psuedotumor cerebrii

A

Acetazolamide
weight loss
severe- steroids till decompression

52
Q

most common neuromuscular congenital disease?

heritance? and gene

A

Duchanne
(heraditery muscular dystrophy)

X-linked- dystrophin gene

53
Q

ילד בין 4 מגיע עם קושי בריצה ובעלייה במדרגות, בבדיקה הפיזקלית נראה שיש היפרטרופיה של השוקיים ודלדול יירכיים

האם מספרת שאחיה סבל מסימפטומים דומים.

מהי האבחנה האפשרית?
מהם סיבוכים אפשרי של המחלה?

A

דושן דיסטרופיה

סיבוכים:
קרדיומיופאתיה- 50-80% (לא בקורלציה עם חומרת המחלה)
פגיעה אינטלקטואלית (ללא קורלציה לפגיעה השרירית)
אטרופיה מוחית

54
Q

Duchenne muscle dystrophy.

which lab reasults rule out this Dgx?

A

Normal levels of CPK

55
Q

Dgx of Duchhene dystrophy?

A

PCR for dystrophenne gene
or
muscle biopsy- and immunohistochemistry for dystrophine

56
Q

Tx for Duchhene?

A

Steroids- משנה מהלך מחלה

57
Q

Dgx and Tx for Mystenia gravis

A

Dgx- EMG, Serology (abs against Ach receptors), Tonsilon test
Tx Pyrostigmine(AchEi).

58
Q

Spinal muscle dystrophy (SMA)
What is the clinical signs?

A

severe hypotonia- prolong head leg
abscence of tendon reflex
fasiciculation (LMN)

pathology in ant.horn of spinal cord

תנועות עיניים שמורות וקוגניציה שמורה, ללא הפרעות סנסוריות (עמודה אחורית)

59
Q

Prognosis of type I SMA w/o Tx?

A

Mortality until age of 2

60
Q

Dgx of SMA (spinal muscular atrophy)

A

Genetic- absence of axon 7 SMN1

in lab- CPK has a minor elevation

61
Q

Tx for SMA ?

A

Genetic Txnosinersen- SMN-ASO
antisense- oligoneculeotide
simisal protein from SMN2&raquo_space; SMN1

62
Q

A new born present to diffuse hypotonia , clubfoot (contracture) and eating and bearthing problems

what is most likley dgx when the mother has the same disease?

A

**Congenital myotonic dystrophy
**
the mother probably have- symptomathic myotonic dystrophy

63
Q

Dgx of gillian barre?
* common pathogens
* LP
* EMG
* Tx
* prognosis (reccurence)

A
  • common pathogens- compylobacter jujuni / mycoplasma
  • LP- high protein
  • EMG- low amplitude and slow rate velocity
  • Tx- IVIG, plasmapheresis
  • prognosis (reccurence)- full resolution. 4% reccurence
64
Q

What is ramsy hunt syndrome?

A

infection with HSV in ear cannal + facialis ipsilateral

65
Q

Tx for bell’s palsy

A

Prednisone for a weak with tapring another week.
sometime acyclovir

must protect the eye with droplet

66
Q

Which medications are in a/w acute dystonic reaction?

A

Haloperidol
pheramin

67
Q

ילדה בת 9 מתייצגת עם טורטיקולויס, הזדקרות הלשון ולרינגוספאזם וטכיפניאה. בריאה ד”כ התלוננה על עצירות ולכן אמא שלה נתנה לה פרמין שהיה בבית

מהי הסיבה האפשרית להתייצגות?

ומהו הטיפול

A

תגובה דיסטונית אקוטית

טיפול- דיפנהידרמין IV

68
Q

most common cause for severe ataxia in kids?

A

drugs

alcohol / anti-epileptics

69
Q

Ataxia telangiectasia is most a/w which types of tumors?

A

Lymphocorticals

  • lymphoma
  • leukemia
  • hodjkin
  • brain tumors
70
Q

eleveted AFP + low IgE, IgA, IgG2, IgG4

is mostly a/w which type of disease?

A

ataxia telangiectasia

71
Q

A kid after simple procedure and sedation is present with rapid elevation of body Tm. on blood semple theres metabolic and respiratory acidosis with high CPK levels.

What is most likley the Dgx? and what is the Tx and how can we prevent it next time?

A

Malignant hyperthermia- AD

Tx- Dentrolene
PPx- dentrolene before sedation

lesion in ryanodin receptor shows after exposure to sedation substance

72
Q

Which Anti-epileptic is a/w hyperamonemia and eleveted in liver enzyme, pancreatitis?

A

Velporate

73
Q

which Anti-epileptic is a/w Steven jhonson syndrome

A

Lamotrigine

74
Q

which Anti-epileptic is a/w kidney stones and glaucomoa and fever

A

Topimarate