Enfermedad de Von Economo: Caso clínico / Lethargic Encephalitis: Report of one case

Lethargic encephalitis (LE) is a Central Nervous System disorder following an upper respiratory tract infection, characterized by sleep disturbances, clinical symptoms corresponding to basal ganglia involvement and in some cases, neuropsychiatric sequelae. We report a 18-year-old mole with a history of sinusitis treated with azithromycin, two weeks before, presenting with fever, headache, confusion and myoclonus. Urine analysis was positive for cannabis. Cerebro spinal fluid analysis showed mononuclear pleiocytosis (109xmm³) and an increase in protein concentration ofl.6 g/dl. Forty eight hours after admission, the patient required mechanical ventilation and subsequently a status epilepticus appeared. Ten days later, fever, rigidity and resting tremor appeared. A magnetic resonance imaging showed hyperintensities in FLALR sequence in the right insular cortex. The patient continued with extreme rigidity, catatonia and mutism. Considering the possibility ofa LE, methyl prednisolone 1 g/day was administered for five consecutive days followed by prednisone 40 mgl day, observing a dramatic improvement of rigidity and tremors.

Mieloma múltiple y arteritis de células gigantes sin amiloidosis / Simultaneous multiple myeloma and giant cell arteritis without systemic amyloidosis

La amiloidosis sistémica primaria y el mieloma múltiple con amiloidosis primaria se han presentado con características clínicas e histopatológicas que simulan una arteritis de células gigantes. Hasta el momento la asociación se basaba en el rol antigénico del depósito de amiloide sobre las arterias, desencadenando la respuesta inmune que finaliza con una arteritis. Presentamos el primer caso en la literatura de un paciente con mieloma múltiple y arteritis de células gigantes sin amiloidosis sistémica, sugiriendo una relación patogénica entre estas dos entidades. En el caso de nuestro paciente se descartó la presencia de amiloide en la pared arterial, por lo que proponemos que el estímulo para el desarrollo de la arteritis podría ser una excesiva producción de interleuquina 6 fabricada por las células mielomatosas

Primary systemic amyloidosis with clinical and histopathologic features of giant cell arteritis has already been described. The association of multiple myeloma (with primary amyloidosis) and giant cell arteritis is also known. We present the first case in the literature of a patient with multiple myeloma and giant cell arteritis without systemic amyloidosis, suggesting a pathogenic relationship between the two diseases