Cerebral venous thrombosis following an immunoglobulin-E mediated anaphylactic reaction.

BACKGROUND: Prothrombotic and pro-inflammatory states are known cerebral venous thrombosis risk factors. To date, two cases of venous thrombotic events after immunoglobulin-E mediated anaphylaxis have been reported. Herein, we describe the first case of cerebral venous thrombosis in close temporal relation with an immunoglobulin-E mediated anaphylactic event. CASE DESCRIPTION: A 51-year-old female presented with headache, language, and mental disturbance lasting for two days. Two days before the onset, she had undergone a provocative test with deflazacort to study an allergy history; after the test she developed a severe anaphylactic reaction. There were no other comorbidities, and in addition to contraceptive pill, she did not take other medications. On admission the patient was drowsy, with anomic aphasia, inattention and memory impairment. Magnetic Resonance Imaging depicted a left caudate and lenticulo-capsulo-thalamic venous infarct and thrombosis in the deep venous system. The patient was treated with anticoagulation and showed progressive improvement. Neoplastic and pro-thrombotic diseases were excluded. CONCLUSION: The close temporal association between the anaphylactic reaction and cerebral venous thrombosis suggests that anaphylactic reaction could have been a cerebral venous thrombosis precipitating factor. Immunoglobulin-E have been suggested to have prothrombotic activity by stimulating the release of platelet activation factor, thromboxane A2 and serotonin. This case adds on to the available information on possible cerebral venous thrombosis associated conditions.

Cognitive impairment in liver transplanted patients with transthyretin-related hereditary amyloid polyneuropathy.

INTRODUCTION AND AIM: Hereditary transthyretin-related amyloidosis (ATTR-FAP) is characterized by a progressive neuropathy, cardiomyopathy, nephropathy and ocular disease. More than 90% of amyloidogenic transthyretin is produced by the liver; however, this protein is also synthesized in the choroid plexus. Although some patients have transitory neurologic events, the impact on cognition is still unknown. The aim was to study the cognitive performance of ATTR-FAP V30M patients with long disease course. METHODS: A prospective observational study of a consecutive sample of patients with 10 or more years of disease duration was conducted. All patients underwent an extensive neuropsychological evaluation. RESULTS: Sixteen patients were included, with a mean age of 53 years and mean duration of disease of 18 years. All had been submitted to liver transplantation. The functional status was not incapacitating in the majority, with 75% needing at most a stick to walk and 38% still actively working. The neuropsychological evaluation disclosed episodic memory impairments in 31% and executive dysfunction in 25% of patients. CONCLUSIONS: These novel findings suggest that cognitive dysfunction can be a delayed manifestation of hereditary transthyretin-related amyloidosis. The putative relation of cognitive dysfunction with transthyretin-amyloid deposition can provide another model to study the amyloid hypothesis of cognitive impairment.