Ch. 31 Inflammatory CNS Disorders

Question 1

Inflammatory brain disorders that primarily affect the white matter

Answer 1

1) MS
2) Neuromyelitis optica spectrum disorders

Question 2

Rheumatic disorders with secondary involvement of the CNS

Answer 2

1) NP SLE
2) APAS
3) Neuro-Behcet
4) Neurosarcoidosis

Question 3

Important infectious causes of encephalitis, symptoms and imaging findings of which may overlap with idiopathic inflammatory brain disorders

Answer 3

1) Mycoplasma
2) Influenza A
3) Parainfluenza
4) Enterovirus
5) EBV
6) VZV
7) CMV
8) HSV

Question 4

Inflammatory brain disorders

Answer 4

Table 31.1 (p.440)

Question 5

Clinical manifestations of optic neuritis in inflammatory brain disorders

Answer 5

1) Reduced visual acuity
2) Enlarged blind spot or central scotoma
3) Pain with ocular movements
4) Red-green color desaturation

Question 6

Clinical manifestations of spinal cord lesions in inflammatory brain disorders

Answer 6

1) Motor impairment/s
2) Sensory loss (with a localized sensory level)
3) Bowel and bladder impairment
4) Pain with neck flexion (Lhermitte symptom)

Question 7

Clinical manifestations of brainstem inflammation in inflammatory brain disorders

Answer 7

1) Internuclear ophthalmoplegia
2) Hypersomnolence (d/t diencephalic involvement
3) Hiccups
4) Hyperphagia

Question 8

Clinical manifestations of cerebellar involvement in inflammatory brain disorders

Answer 8

1) Impaired gait balance
2) Dysmetria
3) Dysarthria
4) Impairment of rapid alternating movements

Question 9

Clinical manifestations of cerebral lesions in inflammatory brain disorders

Answer 9

1) Focal motor or sensory deficits
2) Encephalopathy

Question 10

Clinical manifestations of cortical lesions in inflammatory brain disorders

Answer 10

Seizures

Question 11

Clinical manifestations of basal ganglia involvement in inflammatory brain disorders

Answer 11

1) Dystonia
2) Catatonia

Question 12

NMDA receptor encephalitis and other autoimmune encephalopathies characteristically affect this structure of the CNS

Answer 12

Basal ganglia

Question 13

T/F Most inflammatory brain disorders begin with an acute attack

Answer 13

T

Question 14

Antibodies that identify children with neuromyelitis optica spectrum disorder (NMOSD)

Answer 14

Antibodies directed against astrocytic water channel aquaporin 4 (AQP4)

Question 15

Antibodies common in children with ADEM and in children with AQP4-negative NMOSD

Answer 15

Antibodies against myelin oligodendrocyte glycoprotein (MOG)

Question 16

CSF findings seen in infection that are also seen in patients with small vessel vasculitis and in some patients with antibody-associated encephalopathy

Answer 16

WBC count and protein

Question 17

T/F CSF profiles are typically normal in medium-large-sized vessel vasculitis

Answer 17

T

Question 18

T/F Elevated opening pressure is seen in majority of children with CNS vasculitis

Answer 18

T

Question 19

Hallmark of multiple sclerosis (CSF finding)

Answer 19

Presence of oligoclonal bands (intrathecal synthesis of Ig)

Question 20

Inflammation of the vessels of the brain that is NOT associated with vasculitis of any other organ

Answer 20

Primary CNS angiitis

Question 21

Diagnosis of cPACNS is commonly based on

Answer 21

Calabrese criteria proposed for adult PACNS, further defined by size of involved vessels

Question 22

Vessels involved in small-vessel vasculitis cPACNS

Answer 22

Vessels throughout the brain and meninges that are NOT primary branches of the Circle of Willis

Question 23

Vessels involved in large-vessel vasculitis cPACNS

Answer 23

Major arteries such as
1) anterior, middle, and posterior cerebral arteries
2) Vertebral arteries
3) Cerebellar arteries

Question 24

___% of arterial ischemic strokes (AIS) in children are thought to be related to CNS vasculitis

Answer 24

40-60%

Question 25

Boys vs girls: Medium-large vessel cPACNS

Answer 25

Boys

Question 26

Boys vs girls: Small vessel cPACNS

Answer 26

Girls

Question 27

Small vs medium-large vessel cPACNS: Gradual onset of diffuse signs and symptoms

Answer 27

Small

Question 28

Anterior vs posterior vessels: Primary large-vessel angiitis

Answer 28

Anterior vasculature is most commonly affected (up to 30% have anterior and posterior affectation

Question 29

Small vs medium-large vessel cPACNS

Answer 29

Table 31.3 (p.443)

Question 30

Ddx of inflammatory CNS disorders and key features

Answer 30

Table 31.2 (p.442)