. Author manuscript; available in PMC: 2015 Apr 1.

Published in final edited form as: Pediatr Infect Dis J. 2014 Apr;33(4):e99–105. doi: 10.1097/INF.0000000000000237

Table 1.

Characteristics of Previously Reported Pediatric Progressive Multifocal Leukoencephalopathy (PML) Cases in HIV-Infected Children

Case	Reference	Age at PML diagnosis, years	Sex	Initial presentation	CD4 count (cells/mm³) Viral load (copies/mL)	Initial neuroradiology	PML diagnosis	Treatment	IRIS	Outcome

Index		15	F	Dysarthria, fatigue, dysphagia, hemiparesis	CD4=5	MRI: T2 hyperintensities of right cerebellum, midbrain, pons, medulla; vascular ectasia supraclinoid carotid arteries	CSF JCV PCR + Typical histopathology Immunohistochemistry Granule cell neuron infection	cART, steroids	Yes	Died
Index		15	F	Dysarthria, fatigue, dysphagia, hemiparesis	VL=82,000			cART, steroids	Yes	Died

1	⁶	7	M	Left upper and lower extremity weakness, drooling, difficulty eating	CD4=390	MRI: Bilateral white matter hyperintensities of right subcortical/ periventricular region	Typical histopathology In situ hybridization +	AZT	No	Died
1	⁶	7	M		VL=NR		Typical histopathology In situ hybridization +	AZT	No	Died

2	⁸	13	F	Numbness of tongue and chin, sialorrhea, dysarthria, dysphagia, muteness	CD4=7	MRI: Prominent basal ganglia lesions, scattered white matter lesions	CSF JCV PCR + Typical histopathology In situ hybridization +	AZT, interferon	No	Died
2	⁸	13	F		VL=NR			AZT, interferon	No	Died

3	⁸	10	M	Right facial palsy, right hemiparesis, aphasia	NR	MRI: Left frontal lobe lesion	Typical histopathology In situ hybridization +	None	No	Died

4	²²	8	M	Right-sided hemiparesis, aphasia	NR	CT: Tortuous, dilated internal carotid, left middle/anterior cerebral arteries	Typical histopathology	AZT	No	Died

5	⁹	10	M	NR	NR	NR	NR	NR	No	NR

6	⁹	12	F	NR	NR	NR	NR	NR	No	NR

7	¹⁹	13	F	Blurred vision, headache, drooling, dysphagia, dysarthria	CD4=7	MRI: Lesions of the corona radiata, centrum semiovale, basal ganglia, right periatrial white matter	CSF JCV PCR+ Typical histopathology In situ hybridization +	AZT, interferon	No	Died
7	¹⁹	13	F	Blurred vision, headache, drooling, dysphagia, dysarthria	VL=NR		CSF JCV PCR+ Typical histopathology In situ hybridization +	AZT, interferon	No	Died

8	²¹	12	M	NR	NR	NR	Typical histopathology	None	No	Died

9	¹⁰	7	M	Decreased activity, slurred speech, ataxia	CD4=0	MRI: Prolonged T1/T2 relaxation in right cerebellar white matter, peduncle, pons	CSF JCV PCR+ Typical histopathology In situ hybridization+	ddC, steroids, radiation therapy	No	Died
9	¹⁰	7	M	Decreased activity, slurred speech, ataxia	VL=NR		CSF JCV PCR+ Typical histopathology In situ hybridization+	ddC, steroids, radiation therapy	No	Died

10	²³	11	M	Fatigue, headache, unsteadiness, left- cerebellar symptoms	NR	MRI: Focal high T2 signal lesion in cerebellum/brainstem	Typical histopathology	AZT	No	Died

11	¹¹	12	M	Left upper extremity weakness, left low extremity pain	CD4=9.5	MRI: White matter lesions right frontal/parietal/occipital lobes	CSF JCV PCR +	cART	No	Hemiparesis
11	¹¹	12	M	Left upper extremity weakness, left low extremity pain	VL=76,000		CSF JCV PCR +	cART	No	Hemiparesis

12	¹²	13	F	Headache, right hemiparesis	CD4=17	MRI: Hyperintense T2 white matter lesions right cerebellar/pons	CSF JCV PCR +	cART, cidofovir	No	Died
12	¹²	13	F	Headache, right hemiparesis	VL=292,000		CSF JCV PCR +	cART, cidofovir	No	Died

13	¹³	16.5	M	Dysarthria, right facial palsy, drooling	CD4=36	MRI: Diffuse confluent T2 areas of high signal in the bilateral corona radiata	CSF JCV PCR +	cART, cidofovir	No	Cerebellar dysfunction
13	¹³	16.5	M	Dysarthria, right facial palsy, drooling	VL=29,100		CSF JCV PCR +	cART, cidofovir	No	Cerebellar dysfunction

14	¹⁴	12	M	Dizziness, headache, left arm weakness, left cerebellar dysfunction, left facial weakness	CD4=49	MRI: Hyperintense lesions left cerebellar hemisphere, pons, medulla	CSF JCV PCR +	cART, steroids	Yes	Mild cerebellar dysfunction
14	¹⁴	12	M		VL=Undetectable		CSF JCV PCR +	cART, steroids	Yes	Mild cerebellar dysfunction

15	¹⁵	8.5	F	Change in mental status, spasticity of all four limbs, dystonia, seizures	CD4=320	MRI: Asymmetrical subcortical, right frontoparietal, left occipitoparietal/basal ganglia lesions	Radiographic	cART	No	Dystonia
15	¹⁵	8.5	F		VL=NR		Radiographic	cART	No	Dystonia

16	²⁰	6	F	Short term memory loss, myoclonic and generalized seizures	NR	CT: Mineralization of left basal ganglia	CSF JCV PCR – Radiographic	None	No	LTFU

17	¹⁶	22	F	Speech difficulty, right facial drooping, right-sided weakness	CD4=10	MRI: White matter lesions, left ventricular compression of left hemisphere	CSF JCV PCR +	cART, steroids	Yes	Progressive improvement
17	¹⁶	22	F		VL=11,800		CSF JCV PCR +	cART, steroids	Yes	Progressive improvement

18	¹⁷	15.5	M	Dizziness, diplopia, fatigue, depression, ataxia	NR	MRI: Lesion of right cerebellar hemisphere with slight mass effect, right cerebellar peduncle	CSF JCV PCR+ Typical histopathology	cART	No	Died

19	¹⁸	9	M	Right-sided facial palsy, right-sided hemiplegia	CD4=4	CT: Hypodense lesions left frontal lobe with lateral ventricle effacement	CSF JCV PCR+	cART, steroids	Yes	Died
19	¹⁸	9	M	Right-sided facial palsy, right-sided hemiplegia	VL=185,976		CSF JCV PCR+	cART, steroids	Yes	Died

PML, progressive multifocal leukoencephalopathy;

VL, viral load;

CSF, cerebrospinal fluid;

JCV, JC virus;

cART, combination antiretroviral therapy;

IRIS, immune reconstitution inflammatory syndrome;

NR, not reported;

AZT, zidovudine;

ddC, zalcitabine;

LTFU, lost to follow-up