Neuropsiquiatría del síndrome de Susac: a propósito de un caso / Neuropsychiatry of Susac Syndrome: a Case Report

RESUMEN El síndrome de Susac es una entidad clínica poco frecuente, posiblemente mediada por un proceso autoinmune; la tríada clásica se compone de retinopatía, disminución en la agudeza auditiva y síntomas neuropsiquiátricos (encefalopatía). Hay pocos casos descritos con sintomatología neuropsiquiátrica como la sintomatología principal. Presentamos un caso de síndrome de Susac, que corresponde a una mujer de 34 arios, con predominio de sintomatologia neuropsiquiátrica, caracterizada por un síndrome de Klüver-Bucy parcial, un síndrome apático, risa y llanto patológico y alteraciones cognitivas de predominio atencional; dichos síntomas mejoraron cualitativamente con el uso de terapia inmunológica. Este caso revela la importancia de las manifestaciones neuropsiquiátricas como presentación clínica en pacientes con entidades neurológicas.

ABSTRACT Susac syndrome is a rare clinical condition, possibly mediated by an autoimmune process; the classic triad is composed of retinopathy, decreased hearing acuity and neuropsychiatric symptoms (encephalopathy). There are few cases reported with neuropsychiatric symptoms as the main manifestation. We present a case of Susac syndrome in a 34-year-old female with a predominance of neuropsychiatric symptoms, characterised by partial Klüver-Bucy syndrome, apathy syndrome, pathological laughter and crying, and cognitive dysfunction predominantly affecting attention, which showed a qualitative improvement with the use of immunological therapy. This case report highlights the importance of neuropsychiatric manifestations as clinical presentation in patients with neurological conditions.

Clinical analysis of Kluver-Bucy syndrome for 4 cases / 中国神经精神疾病杂志

Objective To improve the cliniciansˊ awareness of Kluver-bucy syndrome (KBS), we retrospectively analyzed 4 cases of KBS such as clinical manifestation, MRI feature, treatment and prognosis. Methods We performed a retrospective analysis on 4 cases of KBS including general clinical data, key clinical symptoms, findings from cerebrospinal fluid and Cranial MRI examination, treatment strategy and prognosis, and literature review. Results All four patients showed KBS in the course of primary disease. The clinical symptoms of KBS mainly presented as gentle, indiscriminate dietary behavior, hypersexuality, hypermetamorphosis, hyperorality and visual agnosia. MRI Showed primary disease-associated alterations in diverse brain regions including hippocampus, the temporal lobe, the insula, the frontal lobe, and the cingulate gyrus. Three cases achieved completely clinical remission after treatment with carbamazepine,fluoxetine and antipsychotic drugs. One patientˊs symptoms disappeared spontaneously and no recurrence of symptoms was observed in the following 6~12 months. Conclusion Bulimia is often the first symptom of KBS. Cranial MRI is the characteristic of the primary disease and has great diagnostic value. Treatment with antiepileptic and antipsychotics can achieve favorable prognosis.

Bilateral Anterior Opercular Syndrome With Partial Kluver-Bucy Syndrome in a Stroke Patient: A Case Report

Bilateral anterior opercular syndrome and partial Kluver-Bucy syndrome are associated with bilateral middle cerebral artery lesions. The combination of these two syndromes has only been reported in a child with limbic encephalitis. In this case, a 44-year-old woman with bilateral middle cerebral artery infarction, which occurred 2 years prior, could walk independently. However, she showed automatic-voluntary dissociation and anarthria with preserved writing skills. She also presented hypersexuality, hypermetamorphosis, and memory disturbances. Here, we report a case of an adult stroke patient who suffered from bilateral anterior opercular syndrome accompanied by partial Kluver-Bucy syndrome.

Bilateral Anterior Opercular Syndrome With Partial Kluver-Bucy Syndrome in a Stroke Patient: A Case Report

Bilateral anterior opercular syndrome and partial Kluver-Bucy syndrome are associated with bilateral middle cerebral artery lesions. The combination of these two syndromes has only been reported in a child with limbic encephalitis. In this case, a 44-year-old woman with bilateral middle cerebral artery infarction, which occurred 2 years prior, could walk independently. However, she showed automatic-voluntary dissociation and anarthria with preserved writing skills. She also presented hypersexuality, hypermetamorphosis, and memory disturbances. Here, we report a case of an adult stroke patient who suffered from bilateral anterior opercular syndrome accompanied by partial Kluver-Bucy syndrome.

Kluver-Bucy Syndrome Following Bilateral Posterior Cerebral Artery Infarction

Kluver-Bucy syndrome is defined as a rare neurobehavioral disorder with hyperphagia, hyperorality, hypersexuality, and visual agnosia. This syndrome is usually resulting from bilateral lesions of the anterior temporal lobe including amygdala. Although it could occur after various diseases, stroke is uncommon etiology. We report a patient with Kluver-Bucy syndrome due to bilateral posterior cerebral artery territory infarction.

Relapsing Herpes Simplex Encephalitis Resulting in Kluver-Bucy Syndrome

Relapse of herpes simplex virus (HSV) encephalitis rarely occurs after acyclovir treatment. We experienced a case of relapsing HSV encephalitis in the contralateral temporal lobe, resulting in Kluver-Bucy syndrome, after a full dose acyclovir treatment. Sudden behavioral and emotional changes after HSV encephalitis treatment suggest relapsing HSV encephalitis as well as temporal lobe epilepsy.

Transient Hyperorality during Automotor Seizure in a Patient with Right Temporal Lobe Epilepsy. Epileptic Kluver-Bucy Syndrome? / 대한간질학회지

Kluver-Bucy Syndrome (KBS) is consisting of hyperorality, emotional blunting, hypersexuality, altered dietary habits, visual and auditory agnosia. It has been reported in variable neurological diseases. However, only a few cases reported in epilepsy. We report a patient with unilateral temporal lobe epilepsy who presents transient hyperorality during seizure. A 46-year-old man has complex partial seizures which were abdominal aura followed by hyperorality and hand automatisms. Hyperorality was characterized by putting patient's hand or seizure button into his mouth. Brain MRI demonstrated right hippocampal sclerosis. The interictal and ictal SPECT suggested right temporal lobe dysfunction, and PET showed bitemporal hypometabolism. Rhythmic ictal activities were arising from right temporal region when patient presented hyperorality. We speculate that transient hyperorality in this patient could be a symptom of KBS. This case suggests that transient KBS can be occurred in a unilateral temporal lobe epilepsy when ictal discharges cause bitemporal dysfunction during temporal lobe seizure.

Kluver-Bucy Syndrome (review) / 中国康复理论与实践

@#Kluver-Bucy syndrome (KBS) is a rare neuropsychiatric disorder. The characteristics of KBS include visual agnosia, hyperorality, changes in dietary habits, hypermetamorphosis, placidity, hypersexuality, and etc. However, some features like aphasia, amnesia,dementia and seizure are probably exclusive to humans. KBS is usually associated with lesions of the bilateral temporal lobe.