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Pedia > Other CNS Disorders > Flashcards

Other CNS Disorders Flashcards

1
Q

Acute demyelinating polyradiculoneuropathy (symmetric ascending muscle weakness or paralysis)

Paralysis usually ff a NONSPECIFIC VIRAL INFECTION (GIT or RT) by 10 days (Campylobacter jejuni and herpesvirus)

A

Guillain Barre syndrome

postinfectious polyneuropathy involving mainly motor

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2
Q

Etiology of Guillain Barre syndrome

A

Autoimmune reaction that develops in response to a previous infection – ABERRANT DEMYELINATION of PERIPHERAL NERVES and VENTRAL MOTOR NERVE ROOTS

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3
Q

Landry Ascending Paralysis

A

Weakness begins in the LOWER EXTREMITIES and progressively involves the TRUNK, UPPER LIMBS and BULBAR MUSCLES

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4
Q

Other physical findings associated with GBS

A

CRANIAL NERVE DEFICITS - dysphagia, dysarthria, facial weakness, papilledema, autonomic dysfunction, respiratory muscle paralysis

MILLER-FISHER SYNDROME - acute ophthalmoplegia, ataxia, areflexia

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5
Q

The last function to resolve in GBS

A

Extremity weakness

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6
Q

The last function to recover in GBS

A

Tendon reflexes

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7
Q

3 Clinical features are predictive of POOR OUTCOME with sequela

A

CN involvement
Need for intubation
Maximum disability at the time of presentation

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8
Q

Laboratory findings in GBS

A

INCREASED PROTEIN
normal glucose
(-) pleocytosis

ALBUMINOCYTOLOGIC DISSOCIATION - dissociation b/w high CSF protein

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9
Q

Management of GBS

A

admit - observation

IVIG - rapidly progressive ascending paralysis

supportive care

Methylprednisolone (high dose pulse (V) - relapses

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10
Q

headache, vomiting, decreased activity

new onset falling, stumbling gait

A

MEDULLOBLASTOMA

cerebellum

4-8 y/o

MALE

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11
Q

MC malignant brain tumor in children

A

Medulloblastoma

HOMER WRIGHT ROSETTES - circular patterns of tumor cells surrounding a center of neutrophils

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12
Q

Infratentorial Tumors

A

Cerebellar astrocytoma - MC; best prognosis

Medulloblastoma

Brain stem glioma

Ependymoma

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13
Q

Supratentorial Tumors

A

Craniopharyngioma

Optic nerve glioma

Astrocytoma

Choroid plexus papilloma

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14
Q

Strokes most often involved this artery

A

MCA

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15
Q

Diagnostic of choice in Arterial Ischemic Stroke (AIS)

A

MRI

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16
Q

3 main etiologies of Arterial Ischemic Stroke (AIS)

A

ARTERIOPATHY - disorders of cerebral arteries
CARDIAC - cardioembolic strokes
HEMATOLOGIC - sickle cell anemia (400 fold increased risk of stroke), IDA, coagulation disorders

17
Q

Management of AIS

A

ANTITHROMBOTIC STRATEGIES
Heparin
Aspirin

NEUROPROTECTIVE STRATEGIES
Glucose control
Temperature control
Seizure prevention
Maintenance of cerebral	perfusion

Secondary Stroke Prevention (antiplatelet therapy in arteriopathy and anticoagulation in cardiogenic causes)

Rehabilitation

18
Q

Thrombotic occlusion of venous structures create increased ICP, cerebral edema and venous infarction or hemorrhage

A

Cerebral Sinovenous Thrombosis (CSVT)

19
Q

Diagnostic of choice in Cerebral Sinovenous Thrombosis (CSVT)

A

Contrast CT venography or MR venography

20
Q

Management of Cerebral Sinovenous Thrombosis (CSVT)

A

Anticoagulation therapy (unfractionated or LMWH)

21
Q

The MCC of childhood subarachnoid and intraparenchymal hemorrhagic stroke

A

AV malformation

22
Q

Arise from a DEFECT IN DIFFERENTIATIONOF THE PRIMITIVE ECTODERM

A

Neurocutaneous Syndrome

23
Q

AD trait

Heterogeneous disease with a wide clinical spectrum varying
from severe MR & incapacitating seizures to normal intelligence and a lack of seizures, often within the same family

Affects many organs like skin, brain, heart, kidney, eyes, lungs, bone

A

Tuberous Sclerosis

24
Q

Mutated genes in Tuberous Sclerosis

A

TSC1 – hamartin
TSC2 - tuberin

tumor suppressor genes

25
Q

Characteristic brain lesions in Tuberous Sclerosis

A

TUBERS (subependymal region) – undergo calcification and project into the ventricular cavity – CANDLE-DRIPPING APPEARANCE

26
Q

Clinical Manifestations of Tuberous Sclerosis

A

Infantile spasms
ASH LEAF

generalized seizure
SHAGREEN PATCH (lumbosacral region) - roughened, raised lesion with an orange peel

SUBUNGUAL OR PERIUNGUAL FIBROMA

RHABDOMYOSARCOMA

BILATERAL ANGIOMYOLIPOMAS and CYTS

27
Q

2 types of retinal lesions on tuberous sclerosis

A

mulberry tumors

round gray flay lesions

28
Q

AD
every system may be affected

d.t. ABNORMALITY of NEURAL CREST CELL DIFFERENTIATION and MIGRATION during the early stages of embryogenesis

A

Neurofibromatosis

NF-1
NF-2

29
Q

The most prevalent type of Neurofibromatosis

A

NF-1

diagnosed when any 2/7 of the ff are

  1. 6 or more CAFE 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. 1st-degree relative with NF-1 whose diagnosis was based on the
    aforementioned criteria
  8. Majority of mutations in NF-1 occur in the paternal germline
  9. High incidence of learning disabilities
30
Q

NF-2 diagnosis

A

1 of the ff is present:

  1. Bilateral 8th nerve masses (acoustic neuroma)
  2. Parent, sibling, or child with NF-2 & either unilateral 8th nerve masses or any 2 of the ff: neurofibroma, meningioma, glioma, schwannoma
31
Q

NF-1

A 
cafe au lait macules
axillary or inguinal freckles
lisch nodules
neurofibromas
osseous lesion
optic glioma
32
Q

Pattern of weakness in GBS

A

Ascending symmetric paralysis

33
Q

MC location of brain tumors in children

A

INFRANTENTORIAL

34
Q

MCC of arterial ischemic stroke in children

A

ARTERIOPATHY

35
Q

Diagnostic of choice in cerebral sinovenous thrombosis

A

Contrast CT venography or MR venography

36
Q

Etiology of Brain Abscess

A

embolization d.t. CHD w/ R > L shunts

meningitis

chronic OM and mastoiditis

face and scalp infections

orbital cellulitis

dental infections

penetrating head injuries

VP shunt infections

37
Q

Brain Abscess

S. aureus
Streptococci
Anaerobes
Gram (-) aerobic bacilli (Proteus, Pseudomonas, Haemophilus, Citrobacter)

A

CEREBRUM (80%)

single

38
Q

Diagnostic of Brain Abscess

A

Cranial CT scan and MRI

39
Q

Indications of Surgery (Brain Abscess)

A 
(+) gas in the abscess
multiloculated abscesses
posterior fossa location
fungal cause
associated infections - mastoiditis, periorbital abscess, sinusitis

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