Table 1

Clinical features in patients with VGKC-complex antibodies <400 pM

SubjectHighest VGKC levels; other specific antibody if found*Physician's diagnosis/duration of follow-upCSF (cerebrospinal fluid)Response to immunotherapiesVGKC-complex related syndrome
M, 57398Morvan's syndrome/34 months2 WC; Pro NYesDefinite
M, 72168Morvan's syndrome and CIDP/36 months; ThymomaNTYesDefinite
F, 71124, NTAutoimmune/paraneoplastic cerebellar syndrome; Endometrial carcinoma1 WC, Pro 0.64, cytology, culture negativeNoParaneoplastic
F, 67301/LGI1Neuromyotonia/9 monthsNTNot triedDefinite
F, 69144, NMDAR low, GlyRAmnesia/23 monthsAcellular, Pro N, cytology −veNot triedPossible
F, 64170None made/28 monthsAcellular, Pro 0.7, OCB +ve serum and CSFNot triedPossible
M, 25173Brainstem migraine/6 months<1 WC, 16 RC, Pro 0.43, Culture −veNot triedPossible
F, 22316Idiopathic generalised epilepsy/9 months46 WC, (lymph); 4 RC, Pro 0.46, Culture −ve; OCB +ve CSF onlyNot triedPossible
M, 86358/LGI1 and CASPR2Idiopathic Parkinson's disease/3 monthsNTNot triedPossible
F 40391Myoclonic jerks of undetermined aetiology/7 monthsNTNot triedPossible
M 48201Infective encephalopathy improvement with antibiotics/24 months7 WC (lymph); 0 RC, Pro N, Culture −veNot triedPossible
M, 18117/LGI1 and CASPR2Nerve hyperexcitability syndrome/18 monthsNTNot triedPossible
M, 55100Length dependent sensory axonal neuropathy/13 monthsNTNot triedPossible
F, 38149Suspected muscle channel disease/44 monthsNTNot triedPossible
F, 43212Dystonia of undetermined aetiology/40 months, Breast cancerWC<1, RC 1200, OCB +ve in CSF and serum, Pro 0.7Not triedPossible paraneoplastic
F, 42138Functional movement disorder/51 months, Breast cancerAcellular, Pro N, OCB −veNot triedPossible Paraneoplastic
F, 23186None made, spontaneous improvement/6 monthsAcellular; Pro N; OCB −veNot triedPossible
M, 64143Multi-system atrophy/60 months<1WC, 8 RC, OCB −ve, cytology −veIVIG, no improvementUnlikely
F, 61183Alzheimer's disease/34 monthsNTNot triedUnlikely
F, 75221Lewy Body Dementia/8 monthsAcellular, Pro NNot triedUnlikely
M, 75130Embolic stroke secondary to atrial fibrillation/6 months32 RC, <1 WC, Pro NNot triedUnlikely
M, 74106Alzheimer's disease/12 monthsAcellular, Pro NNot triedUnlikely
M, 54118Motor Neurone disease/24 monthsNTNot triedUnlikely
F, 57119Lewy Body dementia/vascular dementia/24 monthsNTNot triedUnlikely
M, 70162Depression/12 monthsNTNot triedUnlikely
F, 18352Allgrove syndrome/7 monthsAcellular; Pro NNot triedUnlikely
M, 62126Alcohol withdrawalNTNot triedUnlikely
F, 60108Anxiety disorderNTNot triedUndetermined
M, 37118Psychological disorderNTNot triedUndetermined
F, 34119None madeNTNot triedUndetermined
F, 62124None madeNTNot triedUndetermined
M, 38103None madeNTNot triedUndetermined

  • *All available samples were tested for LGI1 and CASPR2; most were not tested for contactin-2. Likelihood of VGKC-complex antibody being related to autoimmunity was determined based on previously published guidelines10 ,11 modified as follows: definite: recognised neurological syndrome and treatment with immunotherapy successful; possible: recognised or possible neurological syndrome and immunotherapy unsuccessful or untried; unlikely: unrelated clinical syndrome and more likely alternative diagnosis; undetermined: unrelated clinical syndrome and no likely relevance. Some samples were not available for retesting (NA).

  • CIDP, chronic idiopathic demyelinating polyneuropathy; LGI1, leucine-rich glioma-inactivated 1; NMDAR, methyl-D-aspartate; N, normal; NT, not tested; OCB, oligoclonal bands; Lymph, lymphocytes; Pro, protein (normal range < 0.65 g/l); RC, red cells; VGKC, voltage-gated potassium channel; WC, white cells.