2019 Volume 56 Issue 3 Pages 93-108
Human papilloma virus vaccination associated neuro-immunopathic syndrome (HANS) is a circumventricular organs dysregulation syndrome, derived from neuro-endocrinological disorders of the hypothalamus and its limbic networks. The average score on the modified Rankin Scale reaches a maximum at 4 years after vaccination. We studied blood, CSF, brain MRI, SPECT, muscle CT, EEG and endocrine tests. We found high blood pyruvates and uric acid, low blood 25 vitamin D hydroxide and LDH, and elevated CSF NSE, which indicates immunosuppression and homeostatic dysregulation of energy metabolism (glycolysis system). Severe HANS patients were diagnosed as sarcopenia by muscle CT (measurement of lower thigh circumference), grip power and BMI, and as osteoporosis by bone mineral quantitative examination. Muscle echograms showed a brightness increase. Brain MRI of a severe HANS patient revealed progress of dilated third ventricle and atrophic cerebral cortex. Hypothalamic disorders were confirmed by abnormal insulin tolerance test results, marked hypoglycemic responses after OGTT, low cortisol levels, and absence of circadian changes in ACTH and cortisol. RI cisternography showed mild degrees of spinal fluid leakage, while a spinal fluid production marker (brain-type transferrin) remained normal. Abnormal results of CVRR and orthostatic examinations indicated both parasympathetic and sympathetic disorders. Decreased reactive hyperemia index scores indicated impaired vascular endothelial function. Pulse wave velocity test results showed increased vascular resistance values for age. The ankle brachial index results were normal. Alpha rhythm abnormalities in EEG indicated thalamic disorders. Decreases in flow at the thalamus and anterior cingulate gyrus in SPECT indicated disorders of the diencephalon and limbic system. We discuss pathogenic mechanism of HANS and its various issues.
