Table 5.

Clinical and diagnostic tests findings, among autoimmune CNS treatment responders

No Age/ sex	Clinical presentation	Abnormal testing	Cognitive testing	Prior IT and outcome	IT trial at Mayo	Measurements improved after IT	Additional IT	Relapses off IT	Follow-up and mRS
1 30 M	Memory loss, behaviour change and hemiparesis (S-L)	PET	STMS 31 NPS abnormal	None	IVMTP	Frequency S-L STMS 31→35 SPECT	MTX (SE) MMF (SE) AZT (SE) >7 y	Yes	12.8 y mRs 1
2 53 F	Memory loss, aphasia, hemiparesis (S-L), loss of motivation and psychosis	MRI EEG	STMS 29	IVMTP and pred Improved	IVMTP and PLEX	STMS 23→31 MRI	Pred and MMF 2 y	Yes	2.6 y mRs 4
4 16 F	Memory loss, hypersomnia, hallucinations and seizures	CSF EEF	STMS 38 NPS normal	Pred, PLEX, IVIG Improved	IVMTP	Seizure frequency EEG	Pred 6 m MMF 2 y	Yes	9.2 y mRs 2
7 53 M	Memory, executive and visuospatial difficulties and behaviour change	PET	STMS 38 NPS abnormal	IVMTP Improved	IVIG	NPS	IVIG 16 m MMF 8 y	Yes	9.8 y mRs1
17 67F	Disorientation, confabulation, personality change, agitation and hallucinations	PET	STMS 31 NPS abnormal	IVMTP, IVIG, PLEX, pred Improved	IVIG and IVMTP	Exam, confusion, agitation and confabulation	Pred and MMF initiated	Yes	2.4 y mRs 4
19 87 F	Disorientation, myoclonus and hypersomnia	EEG PET	STMS 19	None	IVMTP	STMS 19→34	IVMTP 3 m and MMF initiated	No	0.4y mRs 2
20 42 F	Memory loss and hypersomnia	–	STMS 36 NPS abnormal	None	IVMTP	STMS 36→38 NPS	IVMTP 4 m IVIG 2 m (SE) AZT 5 m (SE)	Yes	3.5 y mRs 2
21 69 F	Memory loss	CSF	STMS 18 NPS abnormal	Pred Improved	IVMTP	STMS 18→14 NPS	AZT initiated	Yes	1.8 y mRs 3
22 73 F	Confusion, behaviour change and seizures	MRI CSF EEG PET	STMS 29 NPS abnormal	Pred, IVMTP Improved	IVMTP	STMS 29→36 NPS EEG	IVMTP 10 m	Yes	1.8 y mRs 0
24 30 F	Seizures and memory loss	CSF EEG	STMS 34 NPS abnormal	None	IVMTP	Seizure frequency EEG	IVMTP 3 m and MMF 10 m Relapse—IVMTP 3 m & resume MMF	Yes	3.2 y mRs1
27 56M	Confusion, combative, hallucinations and cranial neuropathies (VII, IX, X)	–	NA	IVMTP, PLEX, pred Improved	IVMTP	Cranial neuropathies and headache	AZT 1 y (SE)	Yes	2.5 y mRs 1
28 67 F	Confusion, behaviour change, mania and psychosis	EEG PET	STMS 35 NPS abnormal	Pred Improved	IVMTP	NPS	MMF initiated	Yes	4.4 y mRs2
30 66M	Confusion, agitation, hallucinations and ataxia	MRI CSF EEG	NA	IVIG PLEX	IVMTP Pred	Exam, hallucinations and encephalopathy	None	No	1.4y mRs 0
32 41 F	Memory, hemiparesis, ataxia	CSF PET	STMS 19	IVMTP, AZT, MMF, RTX Improved	IVMTP	STMS 19→24 PET	RTX continued	Yes	6.1 y mRs 2
35 68 F	Memory loss, aphasia, hallucinations and ataxia	EEG	STMS 31	None	IVMTP	Ataxia and aphasia STMS 31→36	None	No	5.9y mRs2
38 39F	Multiple cranial neuropathies	–	NA	Pred, IVIG Improved	IVMTP	Cranial neuropathies	AZT 3 y IVMTP for relapse CYC initiated	Yes (on AZT)	12.2y mRs1

IT, immunotherapy; STMS, Kokmen short test of mental status; NPS, neuropsychological testing; mRs, modified Rankin score; M, male; F, female; NA, not available; Pred, prednisone; IVMTP, intravenous methylprednisolone; IVIG, intravenous immunoglobulin; PLEX, plasma exchange; MMF, mofetil mycophenolate; AZT, azathioprine; RTX, rituximab; CYC, cyclophosphamide; w, week; m, month; y, year; SE, side effects.