Table 2.
Summary of autoimmune epilepsy studies. Studies are derived from search criteria in methods and details have been included only when they were available in the article. MRI findings: increased T2/FLAIR signal in structures listed within the table otherwise stated and studies showing multiple regions of brain involvement are further described in Supplementary Table 2. AEDs = Antiepileptic drugs; CPS = Complex partial seizures; CLN = Cortical laminar necrosis; CI = Cognitive impairment; DN = Double negative VGKC-complex antibody (without LGI1 or CASPR2 reactivities); EPC = Epilepsia partialis continua; FBDS = Faciobrachial dystonic seizures; FS = Focal seizure; FS+ = Focal seizure with impaired awareness; FM = Figural memory; FU = Follow-up; FLAIR = Fluid attenuated inversion recovery; GTC = Generalized tonic clonic seizures; GS = Generalized seizures; GAD = Glutamic acid decarboxylase; GABA = Gamma amino butyric acid; IT = Immunotherapy; IGE = Idiopathic generalized epilepsy; IVIG = Intravenous immunoglobulins; IgG = Immunoglobulin G; MDZ = Midazolam; MMSE = Mini-mental state examination, MTL = Medial temporal lobe; MTS = Mediotemporal sclerosis; MRI = Magnetic resonance imaging; mRS = Modified rankin score; N/A = Data not available; OCB = Oligoclonal bands; PCPC = Paediatric cerebral performance category scale; PL = Pleocytosis; PLEX = Plasma exchange; PPF = Propofol; ⿿Prot = Protein elevation; SGTCS = Secondary generalized tonic clonic seizure; SPS = Simple partial seizures; SE = Status epilepticus; SIADH = Syndrome of inappropriate ADH secretion; SCLC = Small cell lung cancer; TLE = Temporal lobe epilepsy; TICS = Telephone interview of cognitive status (scored out of 41); VM = Verbal memory.
| Author | Type of study/number of patients/antibody type | Clinical presentation | Types of seizure | EEG | MRI | CSF | AED given before IT? (Number/duration) | Number of AEDs used with IT | IT used/outcome | FU duration (range, median)/drug side effects |
|---|---|---|---|---|---|---|---|---|---|---|
| a. LGI1/CASPR2/VGKC-complex antibody studies | ||||||||||
| Vincent et al., 2004 | Observational n = 1 0 Female = 1 LGI1 (100%, retrospective) |
Amnesia, Seizures, Confusion | GTC (n = 3), FS (n = 3), GTC + CPS/SPS (n = 3) | Normal (n = 2), Diffuse slowing + focal activity (n = 4), Focal activity (n = 2), Diffuse slowing (n = 1), Focal activity + diffuse slowing (n = 1) | Normal (n = 2), Unilateral MTL (n = 3), Bilateral MTL (n = 5) | OCB (n = 5), Mild PL (n = 5) | N/A | N/A | IT:n = 10; steroids, PLEX, IVIG Outcome: Neuropsychology: marked recovery (n = 6), slight recovery (n = 3), no recovery (n = 1) | 2 weeks⿿12 months/N/A |
| Thieben et al., 2004 | Retrospective n = 7 Female = 2 VGKC-complex specificity unknown |
Seizures, Amnesia, Irritability, Apathy | SPS (n = 2), CPS (n = 5), GTC (n = 2), SE (n = 1) | Epileptiform discharges (n = 4), Mild bitemporal slowing (n = 1), Diffuse/generalized slowing (n = 2) | Unilateral MTL (n = 1), Bilateral MTL (n = 6) | OCB (n = 1), ⿿Prot (n = 2) | N/A | N/A | IT: n = 6; steroids. One spontaneous recovery. Outcome. TICS: Good recovery (n = 3; TICS = 34⿿35/41), Partial recovery (n = 3; TICS = 27-33/41), Spontaneous (n = 1; TICS = 36/41)) | 7⿿36 months (median = 24) |
| Mcknight et al., 2005 | Case control n = 16 Female = 9 VGKC-complex specificity unknown |
Amnesia, Seizures, Depression | GTC (n = 9), CPS (n = 7) | N/A | Normal (n = 9), Hippocampal (n = 3), Claustrum (n = 1) | N/A | N/A | N/A | IT:n = 6; steroids and IVIG. Outcome: Good response (n = 5), No response (n = 1) | 2⿿60 months (median = 18) |
| Lai et al., 2010 | Observational n = 57 Female = 2 0 LGI1 (100%) CASPR2 (2%) |
Amnesia, Seizures | Temporal lobe seizures (n = 42), Myoclonus (n = 18) | Epileptiform discharge (n = 4), Diffuse or focal slowing (n = 11), Seizures (n = 11) | Temporal lobe (n = 43) | PL (n = 8), ⿿Prot (n = 13) | N/A | N/A | IT: n = 48; steroids, PLEX, IVIG. Outcome: Full recovery (n = 12), Moderate disability (n = 8), Mild disability (n = 27), Death (n = 3). | |
| Irani et al., 2011 | Observationa n = 29 Female = 10 LGI1 (88%) CASPR2 (12%) Double negative (12%) |
Amnesia, Confusion, Hallucinations, Depression, Dysautonomia, Seizures | FBDS | Normal (n = 9), Diffuse slowing (n = 9), Bilateral frontotemporal slowing (n = 6), Temporal sharp waves (n = 2) | Normal (n = 12), Unilateral MTL (n = 3), Bilateral MTL (n = 10) | Mild PL and ⿿Prot (n = 5) | N/A | 1⿿6 (median = 2.6) | IT:n = 27; steroids PLEX, IVIG and rituximab. Outcome: Reduction in seizure frequency >50% (n = 14), 20⿿50% (n = 12), <20% (n = 1) | 4 years AEDs: Localized rash (n = 8), Erythroderma (n = 2), Steven Johnson syndrome (n = 2) IT: Steroid induced psychosis (n = 1); infection (n = 2) |
| Suleiman et al., 2011 | Case control n = 4 Female = 3 Double negative (100%) |
Encephalopathy, Fever, Behavioural, Seizures | GTC (n = 2), SE (n = 4), GS (n = 2) | Generalized slowing (n = 2), Focal slowing (n = 2), Epileptic activity (n = 1) | Normal (n = 2), Left parietal and bifrontal (n = 1), Cerebral oedema (n = 1) | PL (n = 4), ⿿Prot (n = 2) | N/A | N/A | IT: steroids and IVIG. Outcome: Good recovery (n = 1), TLE + CI (n = 1), TLE + CI + psychiatric (n = 1), CI (n = 1) | 1⿿66 months (median = 15.5) |
| Quek et al., 2012 | Observational n = 32 Female = 19 LGI1 (78%) CASPR2 (6%) Double negative (17%) |
Seizures, Personality changes, Anxiety/Depression | SPS (n = 27), CPS (n = 26), GTC (n = 17), EPC (n = 3) | Epileptiform discharge (n = 20), Focal slowing (n = 13), Electrographic seizures (n = 15), Generalized slowing (n = 12) | Normal (n = 15), Temporal/extratemporal (n = 22) | Normal (n = 11), PL (n = 5), ⿿Prot (n = 17), OCB (n = 5) | >2 for 3 weeks⿿12 years (median = 5) | >2 (median = 3) | IT:n = 27; steroids, PLEX. IVIG. Outcome: Seizure freedom (n = 18), Seizure improvement (n = 4), No change (n = 5) | 3⿿72 months (median = 17) |
| Shin et al., 2013 | Observational n = 14 Female = 6 LGI1 (100%) |
Seizures, Cognitive dysfunction, Dysautonomia | FBDS (n = 10), SE (n = 4) | Epileptiform discharge (n = 8), Focal slowing (n = 2) | Unilateral MTL (n = 4), Bilateral MTL (n = 5) | Normal (n = 10), ⿿Prot (n = 3), PL (n = 1) | N/A | N/A | IT:n = 14; steroids, PLEX, IVIG, rituximab, tacrolimus, cyclophosphamide, azathioprine. Outcome: n = 11 (mRS: 0⿿2), n = 1 (mRS >2); relapse (n = 2) | 1⿿24 months (median = 4.5) |
| Toledano et al., 2014 | Observational n = 12 Female = 5 LGI1 (92%) CASPR2 (8%) |
Seizures | FS (n = 6), FBDS (n = 4). FS+ (n = 10). GTC (n = 7) | Normal (n = 1), Epileptiform discharges (n = 6), Focal slowing (n = 2), Excessive beta activity (n = 1), Temporal activity (n = 1), Extratemporal discharge (n = 1) | Normal (n = 2), Bilateral MTL (n = 3), Right MTL (n = 1), Left MTL (n = 1), Right MTS (n = 1) | Normal (n = 5), ⿿Prot (n = 6), PL (n = 1) | >2 (median = 3) | >1 | IT:n = 12; steroids, PLEX, IVIG, mycophenolate mofetil, azathioprine Outcome: Seizure freedom (n = 10); reduced frequency (n = 2) | 2⿿86 months With IT: Steroid induced psychosis; aseptic meningitis |
| Malter et al., 2014 | Retrospective n = 18 Female = 6 LGI1 (50%) CASPR2 (17%) Double negative (33%) |
Amnesia, Confusion, Depression, Seizures, Anxiety | FBDS (n = 2) | N/A | Bilateral MTL (n = 12) Right MTL (n = 2) Left MTL (n = 5) | ⿿Prot (n = 5), PL (n = 2) | N/A | >1 | IT:n = 18; steroids, mycophenolate mofetil Outcome: Seizure free (n = 13); Persistent seizures (n = 5). No deficit in VM + FM (n = 6); Deficit in VM + FM (n = 4); Deficit in FM (n = 5); Deficit in VM (n = 1) | 5⿿70 months (median = 26) Steroid-induced liver failure (n = 1) |
| Newey et al., 2014 | Observational n = 6 Female = 3 VGKC-complex specificity unknown |
Seizures, Altered mental state, Depression. | CPS (n = 1) | N/A | Unilateral MTL (n = 2), Bilateral MTL (n = 1) | Normal (n = 3), ⿿Prot (n = 2), N/A (n = 1) | N/A | >2 | IT:n = 5 (steroids, PLEX, IVIG). Outcome: Complete/partial response (n = 3) | N/A |
| b. NMDAR-antibody studies | ||||||||||
| Dalmau et al., 2007 | Observational n = 12 Female = 12 |
Psychiatric features, Seizures, Movement disorders, Autonomic instability, Reduced level of consciousness | GTC or CPS (n = 11) | Diffuse slowing (n = 7), Generalized slowing/epileptiform activity (n = 3) | Normal (n = 3), Bilateral MTL, (n = 3), Punctate cortical hyperintensites+/⿿meningeal enhancement (n = 5) | PL (n = 14), OCB (n = 3), ⿿Prot (n = 7) | N/A | >2 | IT: steroids, PLEX, IVIG (n = 3); Tumour resection (n = 2); IT and tumour resection (n = 7). Outcome: Full recovery (n = 8, MMSE = 28/30), partial recovery (n = 2, MMSE = 24/30), Death (n = 3) | 7 months⿿6 years |
| Dalmau et al., 2008 | Observational n = 100 Female = 91 |
Psychiatric features, Seizures, Movement disorders, Dysautonomia, Hypoventilation | GTC (n = 45), CPS (n = 10), SE (n = 6), FS (n = 7), SGTCS (n = 8), EPC (n = 2), Myoclonus (n = 9) | Slow activity (n = 71), Epileptic discharge (n = 21) | MTL (n = 22) | PL (n = 91), ⿿Prot (n = 32), OCB (n = 26) | N/A | N/A | IT:n = 92 (steroids, PLEX, IVIG, cyclophosphamide, rituximab, azathioprine); Tumour resection (n = 51). Outcome: Full recovery (n = 47; mRS = 0; MMSE = 29⿿30), Mild stable deficit (n = 28 mRS = 1⿿2; MMSE = 25 ⿿28) Severe deficit (n = 18), Death (n = 7) | 1⿿194 months (median = 17) |
| Iizuka et al., 2008 | Observational n = 4 Female = 4 |
Psychiatric symptoms, Seizures, Dyskinesia, Hypoventilation | Convulsive seizure (n = 2), Tonic (n = 1) | Diffuse delta activity no Paroxymal discharge (n = 3), Irregular slowing no paroxysmal discharge (n = 1) | Normal (n = 3), MTL (n = 1) | PL (n = 4), OCB (n = 1) | N/A | >2 | IT:n = 2 (steroids, IVIG) Outcome: Full recovery (n = 2); Gradual recovery over 3⿿4 years (n = 2) | 4⿿7 years |
| Niehusmann et al., 2009 | Prospective cohort n = 5 Female = 5 |
Psychiatric symptoms, Extratemporal epilepsy Dyskinesia, Dystonia, Hypoventilation, Reduced consciousness | SGTC (n = 2), CPS (n = 1), SPS + CPS (n = 1) | Focal slowing (n = 4), Generalized slowing (n = 1), Epileptic activity (n = 3) | Normal (n = 2), White matter (n = 1) Cortex (n = 1) | PL (n = 5), OCB (n = 3) | 2 months | N/A | IT: n = 3 (steroids, IVIG); no treatment (n = 2). Outcome: None relapsed on IT; 2 did not relapse without IT | 15⿿36 months (median = 26) |
| Florance et al., 2009 | Observational n = 32 Female = 26 |
Seizures, Behavioural/personality changes, Movement disorders | FS/CPS (n = 19), GTC (n = 2) SE (n = 1), FS/SE (n = 1) | Epileptic activity (n = 7), Focal or diffuse delta/theta (n = 22) | MTL, periventricular and cerebellar (n = 5) | PL (n = 27), ⿿Prot (n = 4), OCB (n = 5) | N/A | >2 | IT: n = 30 (Steroids, PLEX. IVIG, rituximab, cyclophosphamide); Tumour resection (n = 8); Electroconvulsive therapy (n = 2). Outcome: Full recovery (n = 9), Substantial recovery (n = 14), Limited improvement (n = 8) | 2⿿14.4 months (median = 4.5) |
| Irani et al., 2010 | Observational n = 44 Female = 31 |
Seizures, Confusion, Psychiatric symptoms, Behavioural changes, Movement disorders, Dysautonomia | GTC (n = 33) CPS (n = 16), SPS (n = 12) | Epileptiform discharge (n = 22), Generalized slowing in delta or theta range (n = 35) | Normal (n = 34), Hippocampi (n = 4) or white matter (n = 6) | PL (n = 30), OCB (n = 23) | N/A | N/A | IT: n = 35 (steroids, PLEX, IVIG, cyclophosphamide, rituximab, azathioprine, mycophenolate mofetil). Outcome: 75% good recovery (mRS 0 ⿿2) | 3.6⿿121 months (median = 16) |
| Armangue et al., 2013 | Observational n = 20 Female = 14 |
Seizures, Psychiatric symptoms, Movement disorders | Seizures (n = 12) | Generalized slowing (n = 7), Focal slowing (n = 3), Generalized slowing/focal activity (n = 6). Extreme delta brush (n = 1). Epileptiform discharge (n = 1) | Temporal lobe (n = 6) | PL (n = 14), OCB (n = 3), ⿿Prot (n = 1) | N/A | >2 | IT: n = 20 (steroids, PLEX, IVIG, rituximab, cyclophosphamide). Outcome: Full recovery (n = 12, PCPC = 1 or 2), Mild disability (n = 5, PCPC = 1 or 2), Severe disability (n = 2, PCPC = 3 or 4), Death (n = 1) | 4⿿149 months (median = 17.5) |
| Titulaer et al., 2013 | Observational n = 577 Female = 468 |
Seizures, Psychiatric symptoms, Movement disorders, Behavioural changes, Speech problems, Dysautonomia, Reduction in consciousness | Seizures (n = 55) | Slow pattern (n = 398), Epileptic features (n = 115) | Normal (n = 360), Abnormal (n = 180), Unknown (n = 37) | Normal (n = 114), PL (n = 402), ⿿Prot (n = 93), Unknown (n = 45) | N/A | N/A | IT: n = 472 (steroids, PLEX, IVIG, rituximab, cyclophosphamide, and tumour removal); no treatment (n = 29). Outcome: Good in 1st 24 months (n = 394; mRS = 0⿿2), Good in 2nd 24 months (n = 203; mRS = 0⿿2), Poor (n = 29; mRS = 3⿿5), One or multiple relapse (n = 45) | 4⿿186 months (median = 24) |
| Viaccoz et al., 2014 | Observational n = 13 |
Seizures, Amnesia, Dyskinesia, Psychiatry, Cognitive | FS (n = 5). GTC (n = 3) | Normal (n = 2), Slow waves (n = 4), SE/seizure (n = 6) | Normal (n = 6), Hippocampus (n = 4), Occipital (n = 1), Cerebellar (n = 1) | PL (n = 10), ⿿Prot/OCB (n = 4) | N/A | N/A | IT:n = 12 (steroids, IVIG, cyclophosphamide, rituximab, mycophenolate mofetil). Outcome: Favourable (n = 10; mRS = 0⿿1), Complete recovery (n = 6; mRS = 0⿿1), Death (n = 1) | 6⿿44 months (median = 14) |
| Lim et al., 2014 | Observational n = 40 Female = 15 |
Seizures, Psychiatric symptoms, Amnesia, Dysautonomia, Movement disorders | Non- convulsive status (n = 6) | Epileptic discharge (n = 12), Generalized or predominantly frontotemporal slowing (n = 10) | MTL (n = 3) | PL (n = 15), ⿿Prot (n = 13) | N/A | N/A | IT: IT and/or tumour resection (n = 22); No treatment (n = 7). Outcome: Favourable outcome (n = 14; mRS = 0⿿2); Poor outcome (n = 7; mRS = 3⿿6) | 1⿿12 months (median = 4) |
| c. GAD-antibody studies | ||||||||||
| Malter et al., 2010 | Observational n = 9 Females = 7 |
Temporal lobe Seizures | TLE (n = 9) | N/A | Unilateral hippocampal atrophy (n = 3), Bilateral amygdalo-hippocampal signal (n = 3) | OCB (n = 5), PL (n = 2), ⿿Prot (n = 2) | N/A | 2 (1⿿5) | IT: n = 9 (steroids, IVIG, cyclophosphamide). Outcome: Abnormal VM (n = 5); Abnormal FM (n = 4). None seizure free | 18 months |
| Haberlandt et al., 2011 | Retrospective n = 4 Females = 4 |
Amnesia, Depression, Ataxia, Seizures, Cognitive decline | TLE (n = 3). FS (n = 1) | N/A | Unilateral MTL (n = 2), Bilateral (n = 1) MTL | OCB (n = 2) | N/A | N/A | IT: n = 3 (steroids or IVIG). Outcome: Memory impairment + TLE (n = 2), restitution (n = 1), memory impairment + epilepsy (n = 1) | 13⿿67 months |
| Lilleker et al., 2014 | Observational n = 6 Females = 6 |
Seizures | FS + GTCS (n = 5), FS (n = 1) | Focal slowing, sharp and slow waves (n = 6) | Normal (n = 6) | OCB (n = 6), ⿿Prot (n = 1) | 2⿿4 over 9.5 years | >2 | IT: n = 5 (steroids, PLEX, IVIG, azathioprine). Left anterior temporal lobe resection (n = 1). Outcome: No improvement in seizures (n = 4); N/A (n = 1) | Optic neuritis (n = 1); Transverse myelitis (n = 1); Hepatotoxicity (n = 1) |
| d. GABABR-antibody studies | ||||||||||
| Lancaster et al., 2010 | Observational n = 15 Female = 7 | Seizures, Amnesia, Confusion, Psychosis | CPS (n = 2), FS (n = 2), GS(n = 5), GTC (n = 2), SGTC (n = 1). SE (n = 3) | Normal (n = 1). Temporal lobe seizures, Epileptiform discharge or temporal lobe slowing (n = 9). Generalized slowing (n = 2) | Normal (n = 4), MTL (n = 10), Corpus callosum (n = 1) | Normal (n = 1). PL (n = 8), OCB (n = 3) | N/A | N/A | IT: n = 6 (steroid PLEX, IVIG), Tumour resection (n = 3), No treatment (n = 4). Outcome: Good response to IT (n = 6) or IT + Tumour resection (n = 3), No clinical improvement in untreated patients | 3⿿72 months (median = 10) |
| Boronat et al., 2011 | Observational n = 10 Female = 1 |
Seizures, Confusion, Amnesia, Disorientation, Behavioural changes | SE (n = 1) | N/A | Normal (n = 3). Hippocampus and amygdala (n = 7) | PL (n = 4) | N/A | N/A | IT:n = 7 (steroids and/or IVIG), Chemotherapy (n = 4). Outcome: Complete recovery (n = 3), Partial response (n = 4), No response (n = 3) | N/A |
| Hoftberger et al., 2013 | Observational n = 20 Female = 8 |
Seizures, Amnesia, Confusion, Hallucinations, Cerebellar ataxia, Opsoclonus myoclonus | Seizures (n = 17), SE (n = 1) | Normal (n = 5). Epileptic activity with or without generalized slowing (n = 7) | Normal (n = 7), Unilateral or bilateral MTL (n = 9), Pial enhancement (n = 1) | PL (n = 6), ⿿Prot (n = 1), PL/⿿Prot (n = 7) | N/A | N/A | IT: n = 19 (steroids, PLEX, IVIG, rituximab, cyclophosphamide or, mycophenolate mofetil), Chemotherapy (n = 4), No treatment (n = 3). Outcome: Complete recovery (n = 7), Partial recovery (n = 8) | 0.75-45 months |
| Dogan Onugoren et al., 2014 | Retrospective n = 10 Female = 2 |
Amnesia, Confusion, Apraxia, Aphasia, Catatonia, Cerebellar dysfunction | GTC (n = 9), SE (n = 2) | Generalized or focal slowing(n = 6), Epileptic activity without slowing (n = 2), Epileptic activity with focal or generalized slowing (n = 2) | Normal (n = 1), Unilateral MTL (n = 6), Bilateral MTL (n = 3), CLN (n = 3) | PL (n = 6), OCB (n = 7) | N/A | N/A | IT:n = 8 (Steroids, PLEX, IVIG, rituximab, cyclophosphamide, azathioprine), Chemotherapy (n = 4), Radiotherapy (n = 1). Outcome: Full recovery (n = 1), Slight recovery (n = 1), Decline in cognitive function (n = 5), Death (n = 3) | 2⿿9 months |
| e. AMPAR-antibodies studies | ||||||||||
| Lai et al., 2009 | Observational n = 10 Female = 9 |
Amnesia, Confusion, Behavioural changes, Disorientation, Seizures | FS (n = 1), GTC (n = 2) | Normal (n = 2), Slow activity (n = 2), Sharp waves (n = 2), Theta activity (n = 1), Epileptic activity (n = 1) | Normal (n = 1), MTL (n = 8), Changes in the anterior septal nuclei (n = 2) | OCB (n = 3), PL (n = 9), ⿿Prot (n = 7) | N/A | N/A | IT:n = 9 (steroids, PLEX, IVIG, azathioprine), Tumour resection + IT (n = 6). Outcome: Good recovery (n = 9), Relapses (n = 5), Death (n = 1) | 8⿿50 months (median = 16) |
| Graus et al., 2010 | Retrospective n = 4 Female = 4 |
Confusion, Agitation, Personality changes, Aphasia, No seizures | N/A | Diffuse slowing with occasional sharp waves over the frontal region (n = 1), Episodic slow waves in frontal region (n = 1) | Normal (n = 2) | Normal (n = 2) | N/A | N/A | IT: n = 2 (steroids). Outcome: Full recovery (n = 2) | N/A |
| Dogan Onugoren et al., 2014 | Retrospective n = 3 Female = 1 |
Amnesia, Psychiatric symptoms, No seizures | N/A | Normal (n = 3) | Bilateral MTL (n = 3), CLN (n = 2) | PL (n = 1) | N/A | N/A | IT: n = 2 (steroids, PLEX, IVIG, azathioprine, rituximab), IT + chemotherapy (n = 1). Outcome: Good recovery (n = 2), Mild recovery (n = 1) | 5⿿14 months |
| Hoftberger et al., 2015 | Retrospective n = 22 Female = 14 |
Amnesia, Confusion, Insomnia, Seizures | SE (n = 1) | Normal (n = 4) Epileptiform activity (n = 4) Focal activity (n = 5) Generalized activity (n = 5) Slow activity (n = 1) Lateralized periodic slowing (n = 1) | Normal (n = 4), Unilateral MTL (n = 2), Bilateral MTL (n = 9) | Normal (n = 6), PL (n = 11), ⿿Prot (n = 10) | N/A | N/A | IT:n = 25 (steroids, PLEX, IVIG, rituximab, cyclophosphamide), Tumour resection (n = 7), Chemotherapy (n = 8), Radiotherapy (n = 6). Outcome: Good response (n = 5, mRS score, 0⿿1), Partial response (n = 10, mRS score, 2⿿3), Poor response (n = 6) | 5⿿266 weeks (median = 72) |
| Joubert et al., 2015 | Observational n = 7 Female = 4 |
Amnesia, Confusion, Insomnia, Seizures, Cerebellar signs, Tumours | FS (n = 1) | Normal (n = 4) Focal activity (n = 3) | Normal (n = 1) Bilateral MTL (n = 4) Diffuse T2 hyperintensities (n = 2) | Normal (n = 1) OCB (n = 3), PL (n = 5) | 1 | N/A | IT:n = 7 (steroids, PLEX, IVIG, rituximab, cyclophosphamide, azathioprine). Outcome: Good response (n = 3, mRS score = 1), Poor response (n = 3, mRS score = 3), Death (n = 1) | 2⿿31 months (median = 12) |