Table 6.
Twelve patients ‘non-responders’—who received immunotherapy trial, with no improvement and were eventually diagnosed with non-autoimmune diagnosis
| No/Age/ Sex | Onset <3m | Neurological presentation | Prior IT response | NPS abnormal | MRI abnormal | EEG abnormal | AE criteria | Final diagnosis |
|---|---|---|---|---|---|---|---|---|
|
40 79 F |
– | Memory loss, aphasia, apraxia, psychosis and seizures | – | + | Multiple lacunar strokes | Periodic sharp wave complexes | – | Probable Creutzfeldt–Jakob disease |
|
41 48M |
– | Cognitive impairment | – | + | Confluent T2-HI hemispheres and pons | NA | – | Frontotemporal dementia |
|
43 52 F |
– | Aphasia | – | NA | Temporal atrophy (L) | NA | – | Primary progressive aphasia |
|
44 52 F |
– | Aphasia and memory loss | + | + | – | Sharp waves, TIRDA | – | Primary progressive aphasia |
|
45 53 F |
– | Cognitive impairment, hallucinations and aphasia | + | + | – | Atypical spike and wave | – | Probable neurodegenerative disorder |
|
46 71 F |
+ | Sub-acute confusion superimposed on chronic parkinsonism | + | NA | – | Diffuse slowing | HE | Probable neurodegenerative disorder |
|
47 50M |
– | Acute onset tremors and cognitive complaint | + | – | – | – | – | Functional disorder |
|
48 43 F |
– | Cognitive complaint, pain and fatigue | – | + (Mild executive deficits) | – | Bitemporal slowing | – | Fibromyalgia |
|
49 12M |
– | Agitation, fixations (ASD) | + | + | – | – | – | ASD, behavioural |
|
50 22F |
+ | Sub-acute change behaviour and cognition (trisomy 21) | + | NA | – | Diffuse slowing | HE | Trisomy 21, behavioural |
|
51 22M |
– | Cognitive impairment | + | – | – | – | – | Mast cell activation disorder |
|
52 28F |
+ | Sub-acute insomnia, shaking spells and disorientation | NA | – | – | – | HE | Insomnia, spells and mood symptoms |
+, yes; −, no; m, months; ASD, autism spectrum disorder; IT, immunotherapy; NPS, neuropsychological testing; NA, not available; T2-HI, T2-hyperintensity; L, left; AE, autoimmune encephalitis; HE, Hashimoto encephalopathy; TIRDA, temporal intermittent rhythmic delta activity.