Table 1 Clinical manifestations of the patients
| Â | Case #1 | Case #2 | Case #3 | Case #4 | Case #5 | Case #6 | Case #7 | Case #8 | ||
|---|---|---|---|---|---|---|---|---|---|---|
Gender | Female | Male | Female | Female | Female | Male | Female | Male | ||
Age at onset (years) | 49 | 79 | 63 | 57 | 79 | 64 | 58 | 60 | ||
Latency to diagnosis (months) | 2 | 4 | 4.5 | 2 | 0.6 | 0.6 | 4 | 1 | ||
Symptoms | Initial symptoms | Confusion, short-term memory difficulty, speech difficulty, seizures | Decline of short-term memory difficulty, seizures | Confusion, poor memory, seizure | Confusion, seizure | Confusion, speech difficulty, rapid deterioration, obtunded | Confusion, seizure | Seizure, short-term memory difficulty | Confusion, short-term memory difficulty, personality changes, seizures | |
Psychiatric symptoms | Hallucination, paranoia, anxiety | Anxiety | Anxiety, paranoia | Paranoia, anxiety | Hypersomnia | Confusion, hallucination | Hallucination, anxiety, agitation | Confusion, talkative, personality changes | ||
Seizure semiology | Focal dyscognitive seizures | FBDS | FBDS, focal dyscognitive seizures | FBDS | No clinical seizure at onset | Focal dyscognitive seizures, FBDS | Focal dyscognitive seizures | Focal dyscognitive seizures | ||
Pertinent lab study results | LGi1 antibody (serum) | Not tested | +1:1280 | Not tested | + | + | + 1:640 | + | + | |
LGi1 antibody (CSF) | + | + | + | + | + | ꟷ | + | + | ||
Other autoantibodies | AchR antibodies and striated muscle antibodies in serum | Neuronal VGKC in serum | Anti-AchR Ganglionic, anti-TPO in serum | Neuronal VGKC in serum | Neuronal VGKC in serum | GAD65 (low titer), Neuronal VGKC in serum | None | None | ||
Serum sodium (normal 135–155 mmol/L) | 120 | 136 | 118 | 121 | 136 | 117 | 136 | 133 | ||
CSF Results | WBC count (CSF) (normal 0–5/µL) | 4 | 19 | 8 | 3 | 6 | 2 | 3 | 1 | |
Protein (CSF) (normal 15–45 mg/dL) | 46 | 102 | 49 | 22 | 349 | 36 | 32 | 46 | ||
OligoClonal Band (CSF) | Abnormal, type 3 | Normal | Abnormal, type 3 | –- | –- | –- | Normal | Abnormal, type 2 | ||
Brain Imaging | Initial results | FLAIR/T2 hyperintensity seen in the bilateral mesial temporal lobes | No specific findings in MRI, PET showed hypometabolism in bilateral posterior cingulate gyrus and precuneus | Unremarkable | Unremarkable | Diffuse cortical edema, swelling throughout both cerebral hemispheres, left more than right | FLAIR/T2 hyperintensity seen in the left mesial temporal lobes | Unremarkable | Unremarkable | |
Follow up results | Gliosis noted in mesial temporal lobe | Brain atrophy noted | Increased FLAIR/T2 signal in the mesial temporal lobes bilaterally, mild right hippocampal atrophy | Signal changes in the left anterior-medial temporal lobe | Bilateral diffuse cerebral edema, worse in the left temporal regions | Mild left hippocampal atrophy | Hyperintense FLAIR/T2 signal within the bilateral mesial temporal lobes | Increased FLAIR/T2 signal in the mesial temporal lobes bilaterally, mild hippocampal atrophy | ||
EEG | Interictal | Rare epileptiform discharges in the left temporal, focal slowing seen in either temporal region | Normal | Focal slowing in the left temporal regions | Normal | Diffuse slow, lateralized periodic discharges in the left hemisphere | Diffuse slow background recording | Focal slowing in the left temporal regions, L TIRDA | Normal | |
Ictal (vEEG) | –- | No cerebral EEG changes at clinical events | Focal-onset seizures arising from the left temporal regions | Focal slowing in the left temporal regions, no cerebral EEG changes at clinical events | Non-convulsive status | Focal-onset seizures arising from the left fronto-temporal regions | Focal-onset seizures arising from the left temporal regions | Focal-onset seizures arising from the right temporal regions | ||
Tumor identified | Thymoma | None | None | None | None | None | None | None | ||
ASM | Levetiracetam, Lacosamide | Levetiracetam, carbamazepine | Levetiracetam, phenytoin | Levetiracetam, phenytoin | Levetiracetam, valproic acid, Lacosamide, phenytoin | Levetiracetam, Lacosamide | Levetiracetam | Valproic acid, clobazam | ||
Immunotherapy | First line | Methylprednisolone, IVIG, PLEX | IVIG | Methylprednisolone, IVIG | Methylprednisolone, IVIG, PLEX | Methylprednisolone, IVIG | IVIG, Methylprednisolone | Methylprednisolone | Methylprednisolone, IVIG | |
Second line | Rituximab, Cyclophosphamide | None | None | Rituximab | None | Rituximab | None | Rituximab | ||
Time to Immunotherapy (days) | 30 | 120 | 150 | 40 | 20 | 60 | 100 | 30 | ||
Follow up | Follow up duration (months) | 30 | 46 | 60 | 72 | 12 | 10 | 10 | 10 | |
Relapse (months) | Yes (3Â months) | None | Yes (4Â months) | Yes (8Â months) | Yes (2Â months) | Yes (3Â months) | None | Yes (1Â month) | ||
Cognitive assessmenta | Major neurocognitive disorder with deficits in declarative memory and executive function, and persistent poor verbal memory | MOCA 20/30 MMSE 23/30 Mild neurocognitive disorder, Difficulty with delayed recall and visual executive function | Mild neurocognitive disorder, Difficulty with phonemic verbal fluency and impaired graphomotor construction | Moderate neurocognitive disorder, Difficulty with visuospatial abilities, short-term memory, and executive systems function | Severe neurocognitive disorder, MOCA: 0/30 Continued with poor memory; intermittent confusion and in need of maximal assistance for daily physical activities | Mild to moderate neurocognitive disorder tested by CLQT, Difficulty with short-term memory and visual executive function, Intermittent confusion | Moderate neurocognitive disorder tested by CLQT, Difficulty with short-term memory and visual executive function | Moderate to severe neurocognitive disorder, MOCA 9/30, Difficulty with visual executive function, language, memory, and attention, Intermittent confusion | ||
Functionality assessmentb- mRS | Onset | 5 | 1 | 5 | 3 | 5 | 5 | 4 | 5 | |
Last follow-up | 3 | 1 | 3 | 2 | 4 | 2 | 2 | 3 | ||
∆mRS | 2 | 0 | 2 | 1 | 1 | 3 | 2 | 2 | ||
Seizure | No (ASM weaning) | No (ASM weaning) | Continued with refractory focal dyscognitive seizures with reduced frequency | No (ASM weaning) | No (still taking ASMs) | No (still taking ASMs) | No (still taking ASMs) | No (still taking ASMs) | ||