Other CNS Disorders

Question 1

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)

Answer 1

Guillain Barre syndrome

postinfectious polyneuropathy involving mainly motor

Question 2

Etiology of Guillain Barre syndrome

Answer 2

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

Question 3

Landry Ascending Paralysis

Answer 3

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

Question 4

Other physical findings associated with GBS

Answer 4

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

MILLER-FISHER SYNDROME - acute ophthalmoplegia, ataxia, areflexia

Question 5

The last function to resolve in GBS

Answer 5

Extremity weakness

Question 6

The last function to recover in GBS

Answer 6

Tendon reflexes

Question 7

3 Clinical features are predictive of POOR OUTCOME with sequela

Answer 7

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

Question 8

Laboratory findings in GBS

Answer 8

INCREASED PROTEIN
normal glucose
(-) pleocytosis

ALBUMINOCYTOLOGIC DISSOCIATION - dissociation b/w high CSF protein

Question 9

Management of GBS

Answer 9

admit - observation

IVIG - rapidly progressive ascending paralysis

supportive care

Methylprednisolone (high dose pulse (V) - relapses

Question 10

headache, vomiting, decreased activity

new onset falling, stumbling gait

Answer 10

MEDULLOBLASTOMA

cerebellum

4-8 y/o

MALE

Question 11

MC malignant brain tumor in children

Answer 11

Medulloblastoma

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

Question 12

Infratentorial Tumors

Answer 12

Cerebellar astrocytoma - MC; best prognosis

Medulloblastoma

Brain stem glioma

Ependymoma

Question 13

Supratentorial Tumors

Answer 13

Craniopharyngioma

Optic nerve glioma

Astrocytoma

Choroid plexus papilloma

Question 14

Strokes most often involved this artery

Answer 14

MCA

Question 15

Diagnostic of choice in Arterial Ischemic Stroke (AIS)

Answer 15

MRI

Question 16

3 main etiologies of Arterial Ischemic Stroke (AIS)

Answer 16

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

Question 17

Management of AIS

Answer 17

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

Question 18

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

Answer 18

Cerebral Sinovenous Thrombosis (CSVT)

Question 19

Diagnostic of choice in Cerebral Sinovenous Thrombosis (CSVT)

Answer 19

Contrast CT venography or MR venography

Question 20

Management of Cerebral Sinovenous Thrombosis (CSVT)

Answer 20

Anticoagulation therapy (unfractionated or LMWH)

Question 21

The MCC of childhood subarachnoid and intraparenchymal hemorrhagic stroke

Answer 21

AV malformation

Question 22

Arise from a DEFECT IN DIFFERENTIATIONOF THE PRIMITIVE ECTODERM

Answer 22

Neurocutaneous Syndrome

Question 23

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

Answer 23

Tuberous Sclerosis

Question 24

Mutated genes in Tuberous Sclerosis

Answer 24

TSC1 – hamartin
TSC2 - tuberin

tumor suppressor genes

Question 25

Characteristic brain lesions in Tuberous Sclerosis

Answer 25

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

Question 26

Clinical Manifestations of Tuberous Sclerosis

Answer 26

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

Question 27

2 types of retinal lesions on tuberous sclerosis

Answer 27

mulberry tumors | round gray flay lesions

Question 28

AD every system may be affected d.t. ABNORMALITY of NEURAL CREST CELL DIFFERENTIATION and MIGRATION during the early stages of embryogenesis

Answer 28

Neurofibromatosis NF-1 NF-2

Question 29

The most prevalent type of Neurofibromatosis

Answer 29

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

Question 30

NF-2 diagnosis

Answer 30

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

Question 31

NF-1

Answer 31

``` cafe au lait macules axillary or inguinal freckles lisch nodules neurofibromas osseous lesion optic glioma ```

Question 32

Pattern of weakness in GBS

Answer 32

Ascending symmetric paralysis

Question 33

MC location of brain tumors in children

Answer 33

INFRANTENTORIAL

Question 34

MCC of arterial ischemic stroke in children

Answer 34

ARTERIOPATHY

Question 35

Diagnostic of choice in cerebral sinovenous thrombosis

Answer 35

Contrast CT venography or MR venography

Question 36

Etiology of Brain Abscess

Answer 36

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

Question 37

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

Answer 37

CEREBRUM (80%) | single

Question 38

Diagnostic of Brain Abscess

Answer 38

Cranial CT scan and MRI

Question 39

Indications of Surgery (Brain Abscess)

Answer 39

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