Table 1 Clinical and laboratory features of four patients with VGKC-Ab-associated LE
Patient 1Patient 2Patient 3Patient 4§
Age, sex46, female72, male62, female57, male
Presenting symptomsConfusion, hallucination, anorexia, weight loss, somnolenceConfusion, generalised seizures, memory loss, mood swings, somnolenceConfusion, complex partial seizuresMemory loss, generalised seizures, complex partial seizures, somnolence
Preceding history (duration)Neuropathic pain (10 weeks)Generalised seizures (5 months)Memory loss (9 years)? Seizures (12 months)
Other symptomsSweatingDrug-induced rash*Abnormal sensations in right face and upper arm
Lowest recorded core body temperature33°C34.5°C34.8°C33°C
Lowest serum sodium recorded (normal: 135–145 mmol/L)Normal129Normal125
Thyroid functionNormalNormalNormalT3–1.93 pg/ml (2.5–4.3 pg/ml) Normal T4, TSH and TRH tests
CSF white cell count<4/mm3<4/mm3Not performed<4/mm3
CSF protein (normal <0.4 g/L)0.69 g/L0.66 g/LNot performed0.61 g/L
MRI brainNormalNormalAbnormalAbnormal
Initial VGKC-Ab titre (pM)86921355912354
MalignancyThymomaNoneNoneNone
EEGDiffuse generalised slowing with no focal epileptiform activityFluctuating bilateral hemisphere dysfunction, worse on the left and deep midline disturbanceBilateral slow waves with excess theta wavesBilateral frontal delta waves and periodic sharp waves from right temporal lobes
  • *Secondary to carbamazepine

  • The lowest sodium level recorded was 119 mmol/L, but this was while on carbamazepine therapy

  • High signal changes in right hippocampus

  • §Patient 4 was previously reported without VGKC antibody determination,6 and the presence of these antibodies was determined subsequently.7 8 The transient drop in T3 level was considered secondary to a hypothalamic dysfunction. Results of other investigations have also been previously described in detail

  • Fluid-attenuation inversion recovery (FLAIR) sequences showed high intensity lesions bilaterally in the amygdalae, hippocampi and hypothalamus