Ch. 31 Inflammatory CNS Disorders Flashcards
Inflammatory brain disorders that primarily affect the white matter
1) MS
2) Neuromyelitis optica spectrum disorders
Rheumatic disorders with secondary involvement of the CNS
1) NP SLE
2) APAS
3) Neuro-Behcet
4) Neurosarcoidosis
Important infectious causes of encephalitis, symptoms and imaging findings of which may overlap with idiopathic inflammatory brain disorders
1) Mycoplasma
2) Influenza A
3) Parainfluenza
4) Enterovirus
5) EBV
6) VZV
7) CMV
8) HSV
Inflammatory brain disorders
Table 31.1 (p.440)
Clinical manifestations of optic neuritis in inflammatory brain disorders
1) Reduced visual acuity
2) Enlarged blind spot or central scotoma
3) Pain with ocular movements
4) Red-green color desaturation
Clinical manifestations of spinal cord lesions in inflammatory brain disorders
1) Motor impairment/s
2) Sensory loss (with a localized sensory level)
3) Bowel and bladder impairment
4) Pain with neck flexion (Lhermitte symptom)
Clinical manifestations of brainstem inflammation in inflammatory brain disorders
1) Internuclear ophthalmoplegia
2) Hypersomnolence (d/t diencephalic involvement
3) Hiccups
4) Hyperphagia
Clinical manifestations of cerebellar involvement in inflammatory brain disorders
1) Impaired gait balance
2) Dysmetria
3) Dysarthria
4) Impairment of rapid alternating movements
Clinical manifestations of cerebral lesions in inflammatory brain disorders
1) Focal motor or sensory deficits
2) Encephalopathy
Clinical manifestations of cortical lesions in inflammatory brain disorders
Seizures
Clinical manifestations of basal ganglia involvement in inflammatory brain disorders
1) Dystonia
2) Catatonia
NMDA receptor encephalitis and other autoimmune encephalopathies characteristically affect this structure of the CNS
Basal ganglia
T/F Most inflammatory brain disorders begin with an acute attack
T
Antibodies that identify children with neuromyelitis optica spectrum disorder (NMOSD)
Antibodies directed against astrocytic water channel aquaporin 4 (AQP4)
Antibodies common in children with ADEM and in children with AQP4-negative NMOSD
Antibodies against myelin oligodendrocyte glycoprotein (MOG)
CSF findings seen in infection that are also seen in patients with small vessel vasculitis and in some patients with antibody-associated encephalopathy
WBC count and protein
T/F CSF profiles are typically normal in medium-large-sized vessel vasculitis
T
T/F Elevated opening pressure is seen in majority of children with CNS vasculitis
T
Hallmark of multiple sclerosis (CSF finding)
Presence of oligoclonal bands (intrathecal synthesis of Ig)
Inflammation of the vessels of the brain that is NOT associated with vasculitis of any other organ
Primary CNS angiitis
Diagnosis of cPACNS is commonly based on
Calabrese criteria proposed for adult PACNS, further defined by size of involved vessels
Vessels involved in small-vessel vasculitis cPACNS
Vessels throughout the brain and meninges that are NOT primary branches of the Circle of Willis
Vessels involved in large-vessel vasculitis cPACNS
Major arteries such as
1) anterior, middle, and posterior cerebral arteries
2) Vertebral arteries
3) Cerebellar arteries
___% of arterial ischemic strokes (AIS) in children are thought to be related to CNS vasculitis
40-60%
Boys vs girls: Medium-large vessel cPACNS
Boys
Boys vs girls: Small vessel cPACNS
Girls
Small vs medium-large vessel cPACNS: Gradual onset of diffuse signs and symptoms
Small
Anterior vs posterior vessels: Primary large-vessel angiitis
Anterior vasculature is most commonly affected (up to 30% have anterior and posterior affectation
Small vs medium-large vessel cPACNS
Table 31.3 (p.443)
Ddx of inflammatory CNS disorders and key features
Table 31.2 (p.442)
