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#DAY4_PEDIATRICS > NEUROLOGY > Flashcards

NEUROLOGY Flashcards

1
Q

Epilepsy – disorder of the brain characterized by an enduring predisposition to generate seizures and by the neurobiological, cognitive, psychological, and social consequences of this condition; ______ unprovoked seizures occurring in a time frame of ____

A

two or more

>24 hrs​

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2
Q

disorder that manifests one or more specific seizure types and has a specific age of onset and prognosis

A

Epileptic syndrome

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3
Q
  • Formerly known as partial seizure
  • initial activation of a system of neurons limited to part of 1 cerebral hemisphere
  • Subdivided into:
    • Focal Seizures without impairment of consciousness (previously known as simple partial seizures)
    • Focal Seizures with impairment of consciousness a.k.a. focal dyscognitive seizures (previously complex partial seizures)
A

Focal Seizures

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4
Q

Clinical and EEG changes indicate synchronous involvement of all or both hemispheres

A

Generalized Seizures

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5
Q

Most common type OF BENIGN EPILEPSY SYNDROMES WITH FOCAL SEIZURES

A

benign childhood epilepsy with centrotemporal spike

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6
Q

Benign Epilepsy with occipital spikes

A
  • Panayiotopoulos type
    • Early childhood
    • Complex partial seizures with ictal vomiting
  • Gastaut Type
    • Later childhood
    • Complex partial seizure, visual auras, migraine headache
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7
Q

Triad of West Syndrome

A

o Infantile epileptic spasms

o Developmental regression

o EEG = hypsarrhythmia (very chaotic and disorganized brain electrical activity with no recognizable pattern)

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8
Q

Lennox-Gastaut Syndrome Triad

A
  • Developmental delay
  • Multiple seizure type: absence, myoclonic, astatic and tonic
  • EEG = 1-2hz spike and slow waves, polyspike bursts in sleep and slow background in wakefulness
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9
Q

starts during the 1 st 2 months of life with severe myoclonic seizures and burst suppression pattern on EEG o usually caused by inborn errors of metabolism

A

Early myoclonic infantile encephalopathy

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10
Q

similar age of onset and EEG but manifests tonic seizures and is usually caused by brain malformations or syntaxin binding protein 1 mutations.

A

Early infantile epileptic encephalopathy (Ohtahara syndrome)

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11
Q

starts as focal febrile status epilepticus or focal febrile seizures and later manifests myoclonic and other seizure types

A

Severe myoclonic epilepsy of infancy (Dravet syndrome)

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12
Q

formerly called ‘petit mal’, involve brief staring spells that usually last for less than 15 seconds; usually resolves 2-5 years after its onset, usually at puberty

A

Absence seizures

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13
Q

1st line of treatment in absence seizures

A

Ethosuximide

Alternative: Lamotrigine and Valproate

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14
Q

Lumbar puncture should be performed in all patients below _______ old for a first febrile seizure.

A

18 months

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15
Q

Local guidelines by Child Neurology Society of the Philippines on a first febrile seizure:

A
  1. Lumbar puncture should be performed in all patients below 18 months old for a first febrile seizure.
  2. For those equal or above 18 months old, LP should be done in the presence of clinical signs of meningitis.
  3. Neuroimaging studies should not be routinely done in children for a first simple febrile seizure.
  4. The use of continuous anticonvulsants is not recommended in children after a first febrile seizure.
  5. The use of intermittent anticonvulsants (whether Diazepam or Phenobarbital) is not recommended for the prevention of recurrent febrile seizures.
  6. EEG should not be routinely requested for in children with a first simple febrile seizure.
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16
Q

WHEN TO DO LUMBAR TAP?

A
  • all infants younger than 6 mo of age who present with fever and seizure or if the child is ill-appearing or at any age if there are clinical signs or symptoms of concern
  • Is an option in a child 6-12 mo of age who is deficient in Haemophilus influenzae type b and Streptococcus pneumoniae immunizations or for whom immunization status is unknown
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17
Q

Most common seizure disorder during childhood

A

SIMPLE FEBRILE SEIZURE

  • usually associated with a core temperature that increases rapidly to ≥39°C.
  • It is initially generalized and tonic-clonic in nature, lasts a few seconds and rarely up to 15 min, is followed by a brief postictal period of drowsiness, and occurs only once in 24 hr.
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18
Q
A
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19
Q

COMPLEX OR COMPLICATED FEBRILE SEIZURE`

A
  • when the duration is >15 min
  • when repeated convulsions occur within 24 hr
  • when focal seizure activity or focal findings are present during the postictal period.
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20
Q

It is defined as continuous seizure activity or recurrent seizure activity without regaining of consciousness lasting for more than 5 min as part of an operational definition put forth within the past few years.

A

STATUS EPILEPTICUS

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21
Q

strongest evidence for initial and emergent therapy FOR STATUS EPILEPTICUS is

A

diazepam or lorazepam, followed by Phenytoin/Fosphenytoin and phenobarbital, then valproate and levetiracetam

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22
Q

In patients developing febrile status epilepticus, what viral infection is most commonly associated in one third of the cases?

A

HHV-6 and HHV7 (ROSEOLA)

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23
Q

Major and minor risk factors for recurrence of Febrile seizures

A
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24
Q

risk factor with the highest risk of occurence of subsequent epilepsy after a febrile seizure.

A

Neurodevelopmental abnormalities

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25
Q

Syntaxin binding protein

A

Ohtahara Syndrome

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26
Q

• MECP2, CDKL5 and protocadherin 19

A

Rett Syndrome

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27
Q

ARX gene; polymerase G

A

West Syndrome

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28
Q

SCN1A

A

Dravet Syndrome

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29
Q

Triad of Imaging Findings in TB Meningitis?

A
  • Hydrocephalus
  • Basal Enhancements
  • Infarcts
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30
Q

Where is CSF produced?

A

o Choroid plexus epithelium within the cerebral ventricles

31
Q

CSF Flow

A

Choroid Plexus

Lateral Ventricle

Foramen of Monro (Interventricular foramen)

Third Ventricle

Aqueduct of Sylvius (Cerebral aqueduct)

Fourth Ventricle

Foramina of Luschka

Foramen of Magendie

Superior Sagittal Sinus

Arachnoid Villi

32
Q

How is CSF reabsorbed?

A

Arachnoid villus cells, which are located in the superior sagittal sinus, return CSF to the bloodstream within vacuoles (via a process called pinocytosis)

33
Q

Absolute contraindications to a lumbar puncture are the following:

A
  1. Signs of elevated intracranial pressure (decreased or fluctuating level of consciousness, relative bradycardia and hypertension, focal neurological signs, abnormal posturing, unequal, dilated or poorly responsive pupils, papilledema, and abnormal Doll’s eye movement);
  2. Local infection at desired puncture site; and
  3. Radiological signs (in cranial scan or MRI) of obstructive hydrocephalus, cerebral edema or herniation and the presence of an intracranial mass lesion or midline shift
34
Q

Relative contraindications to LP (lumbar puncture may be done but only after appropriate diagnostic and therapeutic interventions are done):

A
  1. Signs of shock, sepsis or hypotension
  2. Coagulation defects [disseminated intravascular coagulopathy (DIC), platelet count <50,000/mm 3 , and therapeutic use of 
warfarin]
  3. Focal neurological deficit (especially for suspected posterior fossa lesions)
  4. Glasgow coma score < 8
  5. Epileptic seizures
35
Q

Patients presenting with CNS infections

A
36
Q

Indications for surgery in brain abscess

A
  1. (+) gas in the abscess
  2. Multiloculated abscesses
  3. Posterior fossa location
  4. Fungal cause
  5. Assoc. infections like mastoiditis, periorbital abscess, sinusitis
37
Q

absence of meningitis indicate a better prognosis of Meningococcemia. TRUE or FALSE.

A

FALSE

The presence of petechiae for <12 hr before admission, absence of meningitis, and low or normal erythrocyte sedimentation rate indicate rapid, fulminant progression and poorer prognosis.

38
Q
  • Asymptomatic colonization to fulminant sepsis
  • Infections of the GUT, conjunctiva, pharynx, CNS, heart, skin, adrenals
  • Diffuse adrenal hemorrhage without vasculitis, DIC, coma, and death - Waterhouse-Friderichsen syndrome (fulminant cases)
A

MENINGOCOCCEMIA

39
Q

DRUG OF CHOICE for MENINGOCOCCEMIA

A
  • Penicillin G 250,000-300,000 U/kg/day IV in 4-6 divided doses for at least 5-7 days (DOC)
  • Ceftriaxone IM or IV (100 mg/kg/day once or twice a day) or Cefotaxime IM or IV (200-300 mg/kg/day every 6 or 8 hours) o * clinical improvement within 24-72 hrs
40
Q

PATIENT PRESENTING WITH CNS INFECTIONS

A
41
Q

What do you give to personnel exposed to Meningococcemia?

A
  • Children: Rifampicin 10 mg/kg po every 12 hrs for a total of 4 doses (max 600 mg); 5 mg/kg/dose for <1 month old
  • Or Ceftriaxone 125 mg single dose IM for < 12 yrs old
  • > 18 yrs old: Ciprofloxacin 500 mg po as a single dose
42
Q

The recommended dose of folate to prevent NTDs during the first pregnancy:

A

400 micrograms or 0.4 mg of folic acid

* succeeding pregnancies or recurrent cases of NTDs: 4000 micrograms or 4 mg of folic acid

43
Q
A
44
Q

the most severe form of neural tube defect (dysraphism)

A

MENINGOCELE

45
Q

What parts are affected in MYELOMENINGOCELE?

A

o Dysfunction of the skeleton, skin, GUT, PNS, CNS

o May be located anywhere along the neuroaxis (lumbosacral 75%)

46
Q

o flaccid paralysis of the LE

o absence of DTRs

o lack of response to pain & touch

o hip subluxation

o clubfeet

o bowel & bladder incontinence

o associated with hydrocephalus (type II Chiari)

A

MYELOMENINGOCELE

47
Q
  • failure of closure of the anterior neuropore
  • 50% assoc with polyhydramnios
A

Anencephaly

48
Q

Absence of cerebral convolutions; smooth brain on CT/MRI

A

Lissencephaly (Agyria)

49
Q

What are the features of migraine?

A

Recurrent headache with symptom-free intervals & at least 3 of the ff: (F-R-U-A-N-T)

  1. (+) Family history
  2. Relief following sleep
  3. Unilateral location
  4. Associated aura
  5. Abdominal pain
  6. Nausea & vomiting
  7. Throbbing in character
50
Q

How do you explain the aura in migraine?

A

Cortical spreading depression (CSD) – a phenomenon associated with high CNS hydrogen & potassium ions with the release of glutamate & nitrous oxide → leads to excitation of trigeminalvascular system → activates the release of VIP → vasodilation → extravasation of plasma proteins from the dural vessels → localized inflammation of dural vessels → excitation of pain sensitive receptor → pain

51
Q

Arise from a defect in differentiation of the primitive ectoderm

A

NEUROCUTANEOUS SYNDROMES

  • Tuberous sclerosis
  • Neurofibromatosis
52
Q

Genes mutated in TUBEROUS SCLEROSIS

A

Tuberous sclerosis complex gene 1 (TSC1) and gene 2 (TSC2)

*TSC1 encodes for the protein hamartin and TSC2 encodes for the protein tuberin.

53
Q
A
54
Q

These skin lesions are associated with what disease?

A

Tuberous Sclerosis

  • Typical hypopigmented skin lesions: ash leaf
  • shagreen patch – roughened, raised lesion with an orange-peel consistency located primarily in the lumbosacral region
  • Adolescence: subungual or periungual fibromas from the fingers & toes
55
Q
A
56
Q

Von Recklinghausen disease

A

NEUROFIBROMATOSIS

  • AD disorder; every system may be affected; complications may be delayed for decades
  • Result of an abnormality of neural crest differentiation & migration during the early stages of embryogenesis
57
Q

Von Recklinghausen disease is located on wat chromosome number

A

chromosome 17

58
Q

NEUROFIBROMATOSIS 1

A

NF-1: most prevalent type: diagnosed when any 2/7 of the ff are present:

  1. 6 or more café au lait macules >5mm in diameter in prepubertals & >15mm in postpubertal individuals: present at birth & increase in size, number & pigmentation with predilection for the trunk & extremities with sparing of the face
  2. Axillary or inguinal freckling consists of multiple hyperpigmented areas 2-3 mm in diameter
  3. 2 or more iris Lisch nodules (hamartomas located within the iris)
  4. 2 or more neurofibromas (along the skin, PNS, blood vessels & within viscera) or one plexiform neurofibroma
  5. Distinctive osseous lesion
  6. Optic glioma
  7. 1 st -degree relative with NF-1 whose diagnosis was based on the aforementioned criteria
59
Q

NEUROFIBROMATOSIS 2

A

NF-2 is diagnosed when 1 of the ff is present:

  1. Bilateral 8 th nerve masses (acoustic neuroma)
  2. Parent, sibling, or child with NF-2 & either unilateral 8 th nerve masses or any 2 of the ff: neurofibroma, meningioma, glioma, schwannoma
60
Q

SUMMARY OF NEUROCUTANEOUS SYNDROMES

A
61
Q

Increased protein, normal glucose, normal cells

A

Cytoalbuminocytologic dissociation found in GBS

62
Q
  • Postinfectious polyneuropathy involving mainly motor
  • Not hereditary; affects all ages
  • Paralysis usually follows a nonspecific viral infection (GIT or RT) by 10 days (Campylobacter jejuni and herpesvirus)
  • Weakness begins in the lower extremities & progressively involves the trunk, upper limbs & bulbar muscles (Landry ascending paralysis)
A

Guillain-Barre syndrome (GBS) or acute demyelinating polyradiculo-neuropathy (symmetric ascending muscle weakness or paralysis)

63
Q

Miller-Fisher syndrome is associated with what disease?

A

GBS

* acute ophthalmoplegia, ataxia, areflexia

64
Q

last function to recover & lower extremity weakness last to resolve in GBS

A

Tendon reflexes

65
Q

MANAGEMENT OF GBS

A
  • Acute stage: admit for observation because ascending paralysis may occur within 24 hrs
  • Rapidly progressive ascending paralysis: IVIG (0.4 gms/kg/day for 5 consecutive days)
  • Supportive care; prevention of ulcers
  • High-dose pulse methylprednisolone IV for relapses
66
Q

most common malignant brain tumor in children

A

medulloblastoma

* It accounts for about 20% of primary CNS neoplasms and approximately 40% of all posterior fossa tumors.

67
Q

Homer-Wright rosettes are seen in what brain tumor?

A

MEDULLOBLASTOMA

68
Q

INFRATENTORIAL TUMORS

A
  • Cerebellar astrocytoma – most common & with the best prognosis; cystic; causes hydrocephalus; resection with 90% 5yr survival rate
  • Medulloblastoma – < 7 year old; can spread to extracranial sites; surgery + irradiation; 80-90% 5-yr survival rate
  • Brain stem glioma

• Ependymoma

69
Q

SUPRATENTORIAL TUMORS

A
  • Craniopharyngioma – solid & cystic areas that tend to calcify; short stature; pressure to optic chiasm produces bitemporal visual field defects
  • Optic nerve glioma – decreased visual acuity & pallor of the discs; 25% have neurofibromatosis; hyperalert & euphoric despite being emaciated; invasion of the hypothalamus leads to obesity or DI

• Astrocytoma

• Choroid plexus papilloma

70
Q

The most common cause of arterial ischemic stroke in pediatric population is:

A

Arteriopathy - disorders of cerebral arteries

71
Q

This type of intracranial hemorrhage is almost always associated with trauma:

A

Epidural

72
Q

MCC of childhood subarachnoid and intraparenchymal hemorrhagic stroke

A

AV malformation

73
Q

REsults from defective cleavage of the procencephalon ; associated with midline facial abnormalities - cyclopia

A

Holoprosencephaly

#DAY4_PEDIATRICS (15 decks)

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