Progressive encephalopathy in a Crohn's disease patient on long-term total parenteral nutrition: possible relationship to selenium deficiency

Abstract

Summary:The case of a patient with Crohn's disease complicated by progressive and irreversible encephalopathy, who had been on long-term total parenteral nutrition due to short bowel syndrome, is described. He initially experienced a disturbance ofhis vision, which was followed by various neurological symptoms during the next 3 years. These symptoms rapidly progressed until he finally developed consciousness disturbance. He also manifested erythrocytic macrocytosis, a low serum level of tri-iodothyronine and a high level of thyroxine. His blood levels of various trace minerals and vitamins were normal, except for selenium, which showed extremely low values. In addition, impaired plasma glutathion peroxidase activity was confirmed. After intravenous supplementation of selenium, macrocytosis, tri-iodothyronine and thyroxine values, and glutathione peroxidase activity all became normalized, yet he improved little neurologically.Our case suggests that long-term selenium deficiency may cause progressive and irreversible encephalopathy, and that careful monitoring of this mineral is necessary when an excessive period of total parenteral nutrition is being considered in the clinical setting.

Selenium deficiency presenting visual disturbance and myelopathy

“…Neurological manifestation of selenium deficiency has been characterized by visual disturbance . However, as in the present case and a previous case, myelopathy, often following anemia, can also be a warning sign of selenium deficiency. In contrast, copper deficiency has been recognized by myelopathy rather than visual disturbance, bearing similarities to subacute combined degeneration associated with vitamin B 12 deficiency .…”

“…Therefore, clinical features of selenium deficiency and copper deficiency could partly resemble each other. In contrast to potentially reversible changes of hair, nail beds or anemia, neurological symptoms of selenium deficiency do not generally improve, even after selenium replacement, probably because they develop as a consequence of progression of irreversible neuropathological changes, many years after initiating total parenteral or elemental enteral nutrition lacking selenium . Thus, initiation of selenium supplementation before development of or at the very early stage of neurological symptoms is crucial.…”

Role of trace elements in parenteral nutrition support of the surgical neonate

“…Symptomatic Se deficiency also causes muscle pain, weakness, and myopathy [81]. Other conditions attributed to Se deficiency include macrocytosis, alopecia, pseudoalbinism, growth retardation, and progressive encephalopathy [95][96][97].…”

Management of Crohnʼs Disease in the New Era of Gut Rehabilitation and Intestinal Transplantation

“…40,45,125,126 Accordingly, early gut rehabilitation including transplantation should be considered before the development of overt and subtle irreversible damage to the patient body organs including the central nervous system. [128][129][130][131][132] We strongly believe that intestinal replacement is the best option for CD patients who lost their nutritional autonomy due to recalcitrant disease despite the use of aggressive immunosuppressive and biologic therapy.…”