[P1-141] Repetitive Localized Leptomeningitis: A Case Report with Unique Radiological Features
[Objective] To present the unique clinical and neuroradiological features of a patient with repetitive localized leptomeningitis.
[Case presentation] A previously healthy 14 years old boy was brought into hospital due to generalized tonic seizure. Seizures occurred intermittently over a period of days and gadolinium-enhanced brain MRI showed T2 FLAIR hyperintense lesions on the surface of left frontal cortex. After methylprednisolone pulse therapy, these lesions distinguished and no more seizures had seen. A month later, in the process of reducing oral steroid, he presented different neurological symptoms as disorientation, agitation, and right homonymous hemianopia. Brain MRI revealed left occipitoparietal cortex lesions on enhanced T2 FLAIR imaging. These multiphasic, repetitive localized leptomeningitides were seen at monthly intervals with variety of neurological symptoms such as seizure, cognitive disability, visual disorder, headache, and MRI findings were coincident with each symptom respectively. Blood examinations revealed no abnormalities. In cerebrospinal fluid (CSF), mild pleocytosis and a high level of interleukin (IL)-6 were seen during acute phases. Bacteriological and virological tests were all negative. Steroid therapy was effective on each neurological attack, but despite aftertreatment by oral prednisolone, monthly recurrences were not prevented. Oral colchicine reduced frequency and severity of recurrences.
[Discussion] We suspect that this case is initial manifestation of some forms of neutrophilic disease as typified by neuro-Behcet disease. Although it does not fulfill the diagnostic criterion of neuro-Behcet disease, pleocytosis dominated by neutrophil, a high level of CNS IL-6, good response to steroid therapy, preventive effect by colchicine suggest the similar pathological condition.
[Case presentation] A previously healthy 14 years old boy was brought into hospital due to generalized tonic seizure. Seizures occurred intermittently over a period of days and gadolinium-enhanced brain MRI showed T2 FLAIR hyperintense lesions on the surface of left frontal cortex. After methylprednisolone pulse therapy, these lesions distinguished and no more seizures had seen. A month later, in the process of reducing oral steroid, he presented different neurological symptoms as disorientation, agitation, and right homonymous hemianopia. Brain MRI revealed left occipitoparietal cortex lesions on enhanced T2 FLAIR imaging. These multiphasic, repetitive localized leptomeningitides were seen at monthly intervals with variety of neurological symptoms such as seizure, cognitive disability, visual disorder, headache, and MRI findings were coincident with each symptom respectively. Blood examinations revealed no abnormalities. In cerebrospinal fluid (CSF), mild pleocytosis and a high level of interleukin (IL)-6 were seen during acute phases. Bacteriological and virological tests were all negative. Steroid therapy was effective on each neurological attack, but despite aftertreatment by oral prednisolone, monthly recurrences were not prevented. Oral colchicine reduced frequency and severity of recurrences.
[Discussion] We suspect that this case is initial manifestation of some forms of neutrophilic disease as typified by neuro-Behcet disease. Although it does not fulfill the diagnostic criterion of neuro-Behcet disease, pleocytosis dominated by neutrophil, a high level of CNS IL-6, good response to steroid therapy, preventive effect by colchicine suggest the similar pathological condition.