Table 1.
Abbreviations: T2WI, T2 weighted image. mPSL, methylpredonisolone. PSL, predonisolone. AZP, azathioprine. MONO, monocytes. PMN, polymorphonuclear leukocytes. CYC, cyclophosphamide. MTX, methotrexate. IVIG, intravenous immunoglobulin. nr, not reported.
Cases filled in red were refractory to initial glucocorticoid therapy. Cases filled in blue had good response to initial glucocorticoid therapy. Other patients received no treatment or the results were unknown.5,7,21,27,28,30)
*The clinical course after the second pulse is not shown.
| Year | Age | Sex | Associated neurologic disorders (most patients had fever, headache or meningeal irritation signs) | CSF Leucocytes (/mm 3) | MRI | Treatment | neurological response to initial therapy | neurological response at end of follow up | Outcome | treatment successfully discontinued | |
|---|---|---|---|---|---|---|---|---|---|---|---|
| this case | 58 | M | amnesia, cognitive impairment, emotional liability, urinary incontinence, euphoria |
33 (32 PMN and 1 MONO) |
ventricular enlargement | mPSL 1 g/day 3 days→ PSL 1 mg/kg/day→ +infliximab→PSL →stopped |
transitory | good | alive | yes | |
| 2011 | 9) | 57 | M | generalized seizure, confusion | 700 (MONO 686) |
T2WI high, gadolinium-enhanced |
high dose i.v. mPSL→ high dose i.v. mPSL+CYC →PSL+CYC→ PSL+MTX→infliximab |
transitory | good | alive | no |
| 2011 | 7) | 52 | M | amnesia, gait disorders and urinary incontinence, acalculia | 231 (PMN 161, MONO 69) |
ventricular enlargement | mPSL 500 mg+IVIG 30 g/day 5 days→ PSL 20 mg/day→ Steroid Pulse |
transitory | good or transitory* | alive | no |
| 2009 | 3) | 62 | M | delirium, hallucinations, agitation, disinhibition, cognitive impairment, seizure, disturbed consciousness, recurrent clonic convulsion | 39 (MONO 23) | FLAIR high | iv mPSL 3 days a week 3 weeks→iv mPSL 3 days a week 4 weeks→ PSL 20 mg/day+tacrolimus 3 mg/day |
transitory | worsend | alive | no |
| 2008 | 12) | 51 | M | coordination disorder, distractibility, emotional lability, insomnia, nocturnal myoclonic jerks, perseveration, attention and concentration deficits, confusion, speech latency, word-finding difficulty, myoclonus | 39 (MONO 59) | high signal abnormalities | PSL 80 mg/day→ Cyclophosphamide 150 mg/day |
worsened | worsend | died (after 10 months of neurological onset) | no |
| 2011 | 4) | 73 | M | transitory loss of consciousness, confusion, disorientation, confabulation, aphasia, hallucinations, cognitive impairment | 89 (MONO 89) | FLAIR high, T2 high | mPSL1500 mg 3 days→ 3500 mg→ mPSL po 24 mg/day→ mPSL 1500 mg+ plasmapheresis→+IVIG |
transitory | worsend | died (after 5 month of disease onset) | no |
| 2009 | 11) | 67 | F | bradykinesia, disturbed consciousness, reduced willingness, walking disorder | 73 (MONO 73) | FLAIR high | mPSL 1000 mg/day 3 days→PSL 40 mg/day→ PSL 60–50 mg/day +MTX 6–8 mg/w+CyA 100–200 mg/day |
transitory | worsend | died (after 6 months of neurological onset) | no |
| 1992 | 19) | 73 | F | decreased consciousness, slow in mention, right eyelid paresis, slight unilateral facial weakness | nr | nr | PSL 100 mg/day→ +Cyc 100 mg/day→ PSL 5 mg/day→both stopped |
good | good | alive | yes |
| 2011 | 7) | 44 | M | amnesia, irritated, anxious | 190 (PMN17 MONO 171) | T2WI high | mPSL 200 mg 5 days→ 120 mg/day one week→ PSL 60 mg/day +AZP 100 mg/day→PSL 25 mg/day+AZP 100 mg/day |
good | good | alive | no |
| 2011 | 7) | 44 | F | anxiety, insomnia | 70 (PMN 28 MONO 42) | normal | mPSL 500 mg iv→mPSL 120 mg/day→+AZP | good | good | alive | no |
| 2011 | 8) | 68 | F | dysarthria, disorientation, impaired language function, agraphia | 100 (PMN 33, MONO 67) | high intensity | mPSL 1 g 3 days→ PSL 1 mg/kg/day→10 mg/day |
good | good | alive | no |
| 2010 | 10) | 70 | M | confusion, hallucinations | 38 | T2WI high, gadolinium enhancement | mPSL 1 g 3 days→ PSL 1 mg/kg/day→15 mg/day |
good | good | alive | no |
| 2008 | 13) | 66 | F | bradykinesia, somnolence, urinary incontinence, mutism, disorientation | 90 | T1WI low, T2WI high, FLAIR high | mPSL 1000 mg/day 3 days | good | good | alive | no |
| 2008 | 12) | 68 | M | comprehension problems, emotional lability, confusion, language problems, amnesia, executive dysfunction, visuospatial impairment, mild anomia | 4 (MONO 2) | T2WI high | PSL 80 mg/day | good | good | alive | no |
| 2007 | 14) | 40 | M | confusion, somnolence | 1500 (PMN 1245) | T2WI high, FLAIR high | intravenous steroid therapy | good | good | alive | no |
| 2007 | 26) | 64 | M | amnesia, disorientation, acalculia, reduced willingness | 14 (MONO 14) | T2WI high, FLAIR high | PSL 30 mg po→ PSL 20 mg/day po |
good | good | alive | no |
| 2006 | 15) | 71 | F | confuse, aphasia, weakness of right extremities | 110 (MONO 100) | enhanced | hydrocortisone 200 mg/day→ PSL 60 mg/day→ PSL 20 mg/day |
good | good | alive | no |
| 2004 | 17) | 38 | M | right-side weakness, diplopia, right side hemipregia, with hyperreflexia and clonus at the ankle, confuse | nr | T2WI high | corticosteroid therapy→ PSL 1 mg/kg/day+AZP |
good | good | alive | no |
| 2004 | 2) | 45 | M | confusion, euphoria, hyperactive behavior, disorientation, amnesia, fever, inappropriately jocular affect, disjointed speech, confabulation, attention deficits | 8000 (MONO 7520) | T2WI high | high dose mPSL→ PSL 40 mg/day |
good | good | alive | no |
| 2004 | 16) | 49 | M | disorientation, somnolent, ataxic, disorientation, gait disorder | 145 (PMN 55 MONO 81) | T2WI high | 1 g mPSL 3 days→ PSL 40 mg/day a week→ 20 mg→10 mg/day+ 200 mg hydroxychloroquine per day |
good | good | alive | no |
| 2004 | 1) | 57 | M | amnesia, anxiety, depressive state | 119 (MONO 105) | T2WI high, FLAIR high, gadolinium enhanced | mPSL 1 g/day 3 days 2 course →60 mg/day |
good | good | alive | no |
| 2004 | 2) | 62 | M | acalculia, confusion, euphoria, amnesia | 24000 (MONO 21360) | T2WI high, FLAIR high | methylprednisone pulse→ PSL 40 mg/day po |
good | good | alive | no |
| 1995 | 18) | 36 | M | horizontal diplopia | 5 (5 MONO) | T2WI high, gadolinium enhancement | PSL 20 mg taper over 2 weeks→ 30 mg/day→3 months→ 10 mg/day |
good | good | alive | no |
| 1991 | 20) | 64 | M | change in mental status, hallucination | 100 (2PMN, 96 MONO) | nr | PSL 100 mg iv→ 60 mg/day→ +Cyclophosphamide 125 mg/day |
good | good | alive | no |
| 1983 | 22) | 58 | F | unsteady in walking, confused, hallucination, disorientation, nystagmus, facial weakness | nr | nr | PSL 80 mg/day→ dapson 200 mg/day |
good | good | alive | no |
| 2012 | 6) | 60 | M | acalculia, dyslexia, right left agnosia, mild right hemiplesia |
138 (MONO 128 PMN 10) | FLAIR high, enhanced | PSL iv→PSL po 20 mg | good | good | improved | no |
| 1984 | 21) | 51 | M | left facial weakness, ataxia, dementia, confuse | normal | nr | steroid | no | no | alive | no |
| 2011 | 7) | 54 | M | bipolar disorder, fmemory loss, hallucinations, amnesia, disorientation, insomnia, irritability | 800 (MONO 800) | T2WI high | mPSL 1000 mg iv 3 days→ PSL 80 mg/day+AZP |
nr | good | alive | no |
| 2009 | 5) | 29 | M | nr | 32 (PMN 32) | T2WI high, FLAIR high | oral steroid→ azathioprine+adalimumab |
nr | nr | alive | no |
| 2000 | 28) | 75 | F | tremor | nr | T2WI high | nr | nr | nr | alive | no |
| 2008 | 30) | 61 | M | convulsions, dicreased interest, slurred speech, hallucinations, somnolent, rigidity | 312 (MONO 299) | T2WI high, FLAIR high, gadolinuim enhancement | no treatment (supportive therapy alone) | good | good | alive | no |
| 2006 | 27) | 53 | M | cognitive impairment, difficulties with problem solving, amnesia, uncharacteristically aggressive and abusive behavior, disorientation, psychomotor dysfunction | nr | DWI high | palliative care | no (no treatment) | no (no treatment) | died (after 18 months from onset) | no |