RESUMO
BACKGROUND: Morvan's syndrome is characterized by peripheral nervous system hyperexcitibility (myokymia and neuromyotonia), hyperhydrosis, sleep disorder, limb paresthesias, and encephalopathy. Voltage gated potassium channel antibodies (VGKC abs) are frequently present. Reduplicative paramnesia (RP) has not been reported with this disorder. OBJECTIVE: To describe a patient with Morvan's syndrome presenting with RP. DESIGN: Single case study. PATIENT: A 64-year-old man with several years of myokymia and myoclonus with escalating parasomnia and confusion developed the delusion that a replica of his house and its contents existed 40 mi away. RESULTS: Serum VGKC ab titer was elevated. Neuropsychological testing disclosed executive function and memory deficits. Electromyography demonstrated diffuse myokymia. Treatment with intravenous immunoglobulin and prednisone produced improvement of RP and myoclonus, but not myokymia. CONCLUSION: RP may occur in patients with VGKC ab-associated Morvan's syndrome. Both RP and nervous system hyperexcitability may respond to immunotherapy including intravenous immunoglobulin and corticosteroids.
Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Mioquimia/complicações , Mioquimia/psicologia , Canais de Potássio de Abertura Dependente da Tensão da Membrana/imunologia , Esquizofrenia Paranoide/genética , Esquizofrenia Paranoide/imunologia , Anti-Inflamatórios/uso terapêutico , Autoanticorpos/sangue , Transtornos Cognitivos/tratamento farmacológico , Transtornos Cognitivos/genética , Transtornos Cognitivos/imunologia , Eletromiografia , Humanos , Imunoterapia/métodos , Síndrome de Isaacs/genética , Síndrome de Isaacs/imunologia , Síndrome de Isaacs/fisiopatologia , Masculino , Transtornos da Memória/tratamento farmacológico , Transtornos da Memória/genética , Transtornos da Memória/imunologia , Pessoa de Meia-Idade , Músculo Esquelético/imunologia , Músculo Esquelético/fisiopatologia , Mioquimia/fisiopatologia , Testes Neuropsicológicos , Nervos Periféricos/imunologia , Nervos Periféricos/fisiopatologia , Prednisona/uso terapêutico , Esquizofrenia Paranoide/tratamento farmacológico , Síndrome , Resultado do TratamentoRESUMO
Neuromyelitis optica or Devic's syndrome is an uncommon demyelinating disorder that preferentially attacks the spinal cord and optic nerves. Although it is well described in adults, childhood neuromyelitis optica has rarely been reported in the literature and is frequently misdiagnosed as severe multiple sclerosis. Recently, a serum immunoglobulin G test for neuromyelitis optica has become available which may clarify and accelerate the diagnosis. This report describes a child with recurrent myelitis and an elongated spinal cord lesion who was found to have positive neuromyelitis optica autoantibody. We believe that neuromyelitis optica autoantibody testing should be performed in cases of pediatric transverse myelitis with multiple vertical segments or recurrence.
