RESUMEN
The collective evidence to date suggests that environmental exposure to excessive amounts of manganese (Mn) can cause a neurodegenerative condition known as manganism. It is now also relatively clear that Mn is involved in the pathogenesis of Alzheimer's disease and at least some prion diseases. The potential involvement of Mn in a panel of other neurodegenerative conditions including Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, and Batten disease has been suggested and investigated, but the results to date are somewhat inconclusive. Herein, previously reported experimental studies investigating the involvement of Mn in the pathogenesis of these conditions are narratively reviewed.
Asunto(s)
Esclerosis Amiotrófica Lateral/inducido químicamente , Enfermedad de Huntington/inducido químicamente , Intoxicación por Manganeso , Manganeso/toxicidad , Lipofuscinosis Ceroideas Neuronales/inducido químicamente , Enfermedad de Parkinson Secundaria/inducido químicamente , HumanosRESUMEN
The behavioral effects of ceroid-lipofuscin accumulation, induced by intraperitoneal administration of acetyl-ethyl-tetramethyl-tetralin (AETT) in Wistar rats for 3 months, were examined in the present studies. A significant increase in neuronal ceroidlipofuscin was demonstrated neuropathologically as well as morphometrically. Although the AETT-intoxicated rats showed neither alteration of locomotor activity nor shock sensitivity, a significant impairment of learning ability, especially an acquisition trial in passive avoidance tests, was observed. Results of the present studies indicate the possibility that a diffuse lipofuscin accumulation causes a learning impairment in rats. The results also imply the possibility of a significant role of age-related lipofuscin accumulation in the dementing processes of human especially in the elderly.
Asunto(s)
Reacción de Prevención/efectos de los fármacos , Lipofuscinosis Ceroideas Neuronales/inducido químicamente , Tetrahidronaftalenos/farmacología , Animales , Electrochoque , Fluorescencia , Hipocampo/efectos de los fármacos , Memoria/efectos de los fármacos , Actividad Motora/efectos de los fármacos , Vías Olfatorias/efectos de los fármacos , Ratas , Ratas WistarRESUMEN
Skin biopsy specimens from two lepromatous leprosy patients with dark brown pigmentation who were receiving long-term clofazimine therapy were studied. Ceroid-lipofuscin pigment was demonstrated inside macrophages that contained numerous phagolysosomes. These contained lipids and clofazimine that appeared as electron-lucent vacuoles and a lipofuscin pigment that was electron dense, granular, and lamellated. Although the presence of the drug in tissues contributed to the skin pigmentation, the main cause was a drug-induced, reversible ceroid lipofuscinosis.