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Table 5 Summary of clinical characteristic, clinical outcome, CSF findings, and treatment in published pediatric patients

From: Small vessel childhood primary angiitis of the central nervous system with positive anti-glial fibrillary acidic protein antibodies: a case report and review of literature

Publication

Number of cases and ages

Clinical Characteristics

CSF pleocytosis range (cells/mcl)

Positive Antibodies

Treatment

Brain biopsy performed

Vasculitis noted on biopsy

Clinical outcome

Pediatric Cases

Dubey et al. 2018 [31]

10 children, 3–15 yo

Viral prodrome, ataxia, seizures meningoencephalitis, peripheral neuropathy, autonomic dysfunction

12–159

GFAP in serum and CSF

Steroids

IVIG

Plasmapheresis

Rituximab

No

-

5/10 – response to 1st line therapy 2/10—response to 2nd line therapy

Outcome not indicated for 3 pts

Francisco et al. 2019 [32]

One 6 yo child

Meningoencephalitis, abnormal eye movements, dysautonomia

198, 385

GFAP CSF

Mycoplasma IgG, IgM

Steroids

IVIG

Rituximab

No

-

Normal neuro exam 17 months after initial symptoms, attention problems at school

Handoko et al. 2019 [33]

One 12 yo child

Acute psychiatric decline 12 mo. after herpes simplex meningitis with migraines, memory deficits, impulsivity, hypersexuality, hallucinations

“Normal”

GFAP serum and CSF

Steroids

IVIG

Mycophenolate mofetil

No

-

Waxing and waning neuropsychiatric symptoms, cognitive difficulties in school

Martin et al. 2018 [30]

One 13 yo child

Meningoencephalitis, photophobia, phonophobia, agitation, ataxia, hyperreflexia, dysautonomia

204

GFAP CSF

NMDA-R CSF

Steroids

IVIG

Plasmapheresis

No

-

Ovarian teratoma identified and removed. Drastic improvement in neurologic symptoms after 2 months. Short term memory difficulty

Oger et al. 2020 [34]

Two children, 10 and 16 yo

Meningoencephalitis, gait difficulty, nystagmus, dysautonomia, bladder dysfunction

280–700

GFAP serum and CSF

Steroids

No

-

Complete Recovery with normal brain MRI

Rutatangwa et al. 2020 [35]

One 6 yo child

Meningoencephalitis

198, 385

GFAP CSF

Steroids

IVIG

Rituximab

No

-

Significant improvement with some residual symptoms at 17 mo. follow up

Theroux et al. 2018 [36]

One 15 yo child

Meningoencephalitis, bilateral leg weakness, dizziness, catatonia, dysautonomia

100

GFAP CSF

IVIG

Plasmapheresis

Rituximab

No

-

Difficulty with memory and stamina, independent in all ADLS

Trau and Gallentine 2018 [37]

One 10 yo child

Meningoencephalitis, weakness, ascending paralysis

“Elevated”

GFAP serum

Steroids

IVIG

Plasmapheresis

No

-

Full recovery within one year