Table 1.

Clinical findings, laboratory and MRI data. ^a .

Patient ID/sex	1/m	2/m	3/m	4/f	5/f	6/m	7/m
Decade at onset	5th	6th	6th	5th	7th	6th	4th
Previous history of sarcoidosis	No	Yes: hepatic	No	Yes: pulmonary, lymphadenopathy, Bell’s palsy, axonal sensorimotor polyneuropathy	Yes: pulmonary, hepatic, (supposedly) renal	Yes: pulmonary	No
Interval of PML-associated symptoms and diagnosis	~ 2 months	~ 2 months	~ 1 month	~ 1 month	~ 2 months	~ 1 month	~ 6 weeks
Interval of diagnosis to death (if applicable)	Alive	24 months	3 months	~ 4 months	Alive	Alive	Alive
Clinical information
Motor examination	Brachiofacially accentuated right-sided hemiparesis	Left-sided hemiplegia	Right-sided hemiplegia, initially brachiofacially accentuated	Moderate tetraparesis accentuated on the right side	Initially brachially accentuated left-sided hemiparesis, developed hemiplegia and right-sided high-grade paresis in the course	Alien limb right arm	Right-sided hemiparesis
Deep tendon reflexes	Increased on the right	Increased on the left	Increased on the right	Increased, right > left	Increased on the left	Normal	Increased on the right
Pyramidal signs	None	Left positive Babinski’s sign	Right positive Babinski’s sign	Positive Babinski’s sign on both sides	None	None	None
Sensory deficit	According to paresis	None	Not assessable due to clinical condition	None	None	None	According to paresis
Visual field	Hemianopia to the right (initial symptom)	Hemianopia to the left (initial symptom)	Not assessable due to clinical condition	Normal	Cortical blindness	Normal	Normal
Coordination	Pronounced ataxia in the right arm	Bradydysdiadochokinesia	Not assessable due to clinical condition	Pronounced ataxia in the right arm, bradydysdiadochokinesia	Dysmetria in the right arm	Impaired fine motor skills	Not assessable due to paresis on the right
Disorientation	Fluctuating	Developed in the course of disease	Severe	Developed in the course of disease	Moderate cognitive deficits developed in the course of disease	Moderate in the course of disease	None
Aphasia	Mild expressive aphasia	None	Severe global aphasia	Severe global aphasia	None	None	Moderate primarily expressive aphasia
Dysarthria	None	Developed in the course of disease	Anarthria	Developed in the course of disease	Developed in the course of disease	Developed in the course of disease	Developed in the course of disease
Dysphagia	None	Developed in the course of disease	Severe	Developed in the course of disease	Developed in the course of disease	Developed in the course of disease	None
Severe aspiration	None	Possible	Severe	Severe	Possible	None	None
Lung function
Vital capacity [5.04 l]	4.01	n.d.	2.18	n.d.	n.d.	3.17	n.d.
MRI scan
Initial scan: interval from onset	2 weeks	4 weeks	4 weeks	5 days	7 weeks	4 weeks	8 weeks
Initial scan: findings	T2-hyperintense, confluent, subcortical lesion in the left occipital lobe, no Gd enhancement	T2-hyperintense, confluent, subcortical lesion in the right parietooccipital region, no Gd enhancement	T2-hyperintense, confluent, subcortical lesions in the left frontal and both parietal lobes, no Gd enhancement	10 new supra- and infratentorial T2-hyperintense lesions in both parietal lobes, temporo-occipital left lobe, and frontal right lobe, cortical and subcortical, four of them confluent, affection of subcortical U-fibres	T2-hyperintense confluent cortical and subcortical lesion in the right parietooccipital and temporooccipital region	T2-hyperintense cortical lesion in the left parietal region with additional T2 shine-through effect, affection of U-fibres in the left precentral region	T2-hyperintense confluent cortical and subcortical lesions in the left parietal, frontal, and parietooccipital region with surrounding oedema
Most recent scan: interval from treatment initiation	54 months	2 months	2 months	1 week	16 months	51 months	8 months
Most recent scan: findings	Subcortical sclerosis and asymmetric atrophy in the left occipital lobe, e vacuo extension of the left lateral ventricle	Progressive, bilateral, confluent T2-hyperintense lesions including basal ganglia, punctate Gd enhancement in the left parietal region	Progressive, bilateral, confluent T2-hyperintense lesions involving both hemispheres, diffuse punctate Gd enhancement in the left hemisphere	Progressive, bilateral confluent T2-hyperintense lesions involving both hemispheres, affection of U-fibres, Gd+, progression of cerebellar lesions and lesions in medulla oblongata	Progressive, bilateral and right dominated, confluent T2-hyperintense lesions including right external capsule, thalamus, and pyramidal track, affection of U-fibres, atrophy of the right hemisphere, progressive e-vacuo atrophy of the right lateral ventricle	Confluent, cortical T2-hyperintense lesions in the left hemisphere including thalamus, pons, left pedunculus cerebelli, and pontomesencephal; atrophy in the left hemisphere with e-vacuo extension of the left lateral ventricle	Enlargement of the multifocal, confluent left frontal and parietal lobe lesions
IRIS	No	Yes	Yes	Yes	Yes	No	No
CSF (baseline)
Oligoclonal bands	Negative	Negative	n.d.	Positive	Positive	Negative	Negative,
Protein [0.13–0.4 g/l]]	0.46	1.06	0.35	0.62	0.80	0.78	0.37
Cell count [<4/µl]	1	1	1	1	1	2	1
JC virus count/PCR [0 c/ml]	25787	51	2610	21	8	90	4980
ASI [<1.5]	n.d.	n.d.	n.d.	0.63	0.45	3.31	n.d.
CSF (after 3 months)
Oligoclonal bands	Positive	n.d.	n.d.	Positive	Positive	Negative	Positive
Protein [0.13–0.4 g/l]]	0.58	n.d.	n.d.	0.70	0.70	0.55	0.42
Cell count [<4/µl]	4	n.d.	n.d.	1	1	1	1
JC virus count/PCR [0 c/ml]	555	n.d.	n.d.	n.d.	0	0	220
ASI [<1.5]	13.9	n.d.	n.d.	n.d.	0.45	n.d.	n.d.
Further laboratory findings (baseline)
Overall leucocytes [4200–9100/µl]	5200	3400	8200	6500	4300	5,100	6,800
Lymphocytes absolute [4200–9100/µl]	530	1110	990	1150	630	270	1,000
Lymphocytes relative [22–53%]	10.2	32.5	12.1	16.2	14.4	5.3	16.1
Total T-cell count [1100–1700/µl]	139	1014	983	–	476	83	450
CD4+ T-cell count [645–1289/µl]	106	464	404	–	382	70.9	132
CD8+ T-cell count [263–739/µl]	29	331	282	–	95	42.6	271
EBV (serology)	n.d.	n.d.	Positive (IgG)	n.d.	Negative	Negative	Negative
Quantiferon test	Negative	n.d.	Negative	n.d.	n.d.	n.d.	Negative
ACE [8.28 mU/ml]	24.3	n.d.	n.d.	19.4	72.5	21.5	13.7
sIL2-receptor [223–710 U/ml]]	664	55.6	820	429	3891	458	824
ASAT [<50U/l]	67	33	45	17	42	24	33
ALAT [<50U/l]	102	24	42	17	52	30	30
γ-GT [<60U/l]	215	245	133	21	244	34	25
Therapeutic regimes
Therapy of sarcoidosis before PML	None	None	None	Azathioprine for 2 months, mycophenolate mofetil, infliximab for 3 months, fumaric acid for 3 months	None	Azathioprine 150 mg per day for 2 years	None
Therapy of sarcoidosis after diagnosis of PML	Prednisolone 50 mg 1-0-0 for four weeks, then monthly reduction of 10 mg, containing dosage: 5 mg 1-0-0	Prednisolone 5 mg 1-0-0 for 5 months, stop due to PML progression with immunosuppression	Prednisolone 500 mg 1-0-0 for 3 days, then prednisolone 100 mg 1-0-0 for 7 days, then prednisolone 50 mg 1-0-0 with originally planned monthly reduction of 10 mg, stop due to PML progression with immunosuppression	None	Initially none, Prednisolone 5 mg 1-0-0, 50 mg 1-0-0, slow reduction; containing dosage: 5 mg 1-0-0	None	Prednisolone 40 mg 1-0-0
Medication due to PML	Mirtazapine 45 mg 0-0-1, mefloquine 250 mg 1/week	Mirtazapine 60 mg 0-0-1, mefloquine 250 mg 1/week	Mirtazapine 30 mg 0-0-1, mefloquine 750 mg 1-0-0, then mefloquine 250 mg 1/week	Cidofovir 5 mg/kg bw (250 mg) twice in an interval of 7 days	Mirtazapine 60 mg 0-0-1, mefloquine 250 mg 1/week, cidofovir 5 mg/kg bw for 3 months	Mirtazapine 60 mg 0-0-1, mefloquine 250 mg 1/week, cidofovir 5 mg/kg bw, 13 times, vaccination JCV, IL 2 once 500.000 U/m2 BSA, then for 79 days 1.000.000 U/m2 BSA	Mirtazapine 60 mg 0-0-1 for 5 months, mefloquine 250 mg 1/week (total 4 times), cidofovir 5 mg/kg bw, 15 times for 5 months
Pneumocystis jirovecii prophylaxis	Cotrimoxazole	–	Cotrimoxazole	–	–	–	–
Outcome	Incomplete recovery	Disease progression, death	Disease progression, death	Disease progression, death	Incomplete recovery	Severe disease progression	Incomplete recovery
Residual symptoms	Latent brachial paresis, mild right-sided spasticity with brisk tendon reflexes, ataxia and intentional tremor in the right arm, hemianopia	–	–	–	Tetraparesis with left-sided hemiplegia and mild right-sided paresis, hemihypesthesia, brisk tendon reflexes, improvement of dysphagia	Tetraparesis with high grade brachiofacially accentuated right-sided and mild left-sided hemiparesis paresis, positive Babinski’s sign	Mild right-sided spastic hemiparesis, fully ambulatory, normal speech

^aIf required, the reference values are given in square brackets with the corresponding units. Medications are listed by the drug names and not by the trade names. Based on the availability of information, dosages are not always specified in detail.

γ-GT, gamma-glutamyltransferase; ALAT, alanine aminotransferase; ASAT, aspartate aminotransferase; ASI, antibody-specific index; BSA, body surface area; bw, body weight; c/ml, copies per millilitre; CSF, cerebrospinal fluid; EBV, Epstein-Barr virus; Gd, gadolinium; IL2, interleukin 2; IRIS, immune reconstitution inflammatory syndrome; JCV, JC virus; MRI, magnetic resonance imaging; n.d., not determined; PCR, polymerase chain reaction; PML, progressive multifocal leukencephalopathy; sIL-2, soluble interleukine-2 receptor; U, unit(s).