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.

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.

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.

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.

Kluver-Bucy syndrome--a rare complication of herpes simplex encephalitis.

A twelve-year-old female was admitted with history of high fever, recurrent vomiting and repeated convulsion for 2 days and altered consciousness for one day. Cranial CT scan showed intraparenchymal haemorrhage involving both temporal lobes and right basal ganglia region without mass effect. Serology was reactive against IGM HSV1. Injection acyclovir was started at a dose of 10 mg/kg 8 hourly intravenously. Patient regained consciousness on fourth day but speech was altered. Abnormal behavioural symptoms were noticed. EEG showed generalised spike and slow waves and sharp and slow wave discharge more in the temporal region. The patient was given clonidine and carbamazepine. She also received behavioural therapy and parental counselling. She was followed up for six months and maintaining well.

Kluver-Bucy syndrome -- an experience with six cases.

The Kluver-Bucy syndrome (KBS) is a neurobehavioral syndrome and can be seen in association with a variety of neurological disorders. Case records of 6 patients with KBS seen during a period of 5 years in a university hospital were reviewed. During the study period 6 patients with KBS, aged between 4 and 14 years, were seen. Hyperorality, hypersexuality, and abnormal behavior were the most common manifestations. Of the 6 patients, 5 had recurrent unprovoked seizures. The associated neurological disorders included anoxia-ischemic encephalopthy (2), herpes simplex encephalitis (1), neurocysticercosis (NCC) (1), traumatic brain injury with gliosis (1 case) and tuberculous meningitis (1 case). Prognosis was poor in all the patients except in the patient with NCC.

Herpes simplex encephalitis: some interesting presentations.

Herpes Simplex Encephalitis (HSE) is the most common cause of fatal viral encephalitis. A high index of suspicion is mandatory for early diagnosis and successful therapy to restrict morbidity and mortality. We report 4 patients of HSE, with interesting presentations, viz. brainstem involvement in an immunosuppressed patient, Kluver-Bucy Syndrome-a consequence of untreated HSE, HSE in the postpartum period mistaken as cortical venous thrombosis, and response to inadequate treatment. They demonstrate the wide spectrum of clinical features, pitfalls in diagnosis, and a variable response to therapy in HSE.

Síndrome de Klüver-Bucy por radionecrósis bitemporal / Klüver-Bucy syndrom after radiotherapy with bitemporal necrosis

Se presenta el caso de una mujer de 33 años de edad, a quien se diagnosticó un macroadenoma hipofisiario. Después de realizar dos resecciones quirúrgicas del macroadenoma y de observarse recurrencia, se decidió dar radioterapia con un total de 25 sesiones y dosis acumulada de 5,600 cGy. Dos años después la paciente presentó signos y síntomas del síndrome de Klüver-Bucy (SKB) en relación a encefalopatía posradiación. Este síndrome se presenta cuando existe disfunción temporal bilateral y se caracteriza por alteraciones en la conducta sexual y en los hábitos dietéticos, déficit en la memoria, hiperoralidad, apatía y abulia. Existen reportes escasos en la literatura acerca del SKB en relación a trauma, encefalitis y cirugía; la radionecrósis bitemporal como causa del síndrome es rara, por lo que se considera interesante la presentación de este caso, así como una breve revisión con base a los casos reportados.

Transtorno desintegrativo da infância: relato de dois casos clínicos com estudo de perfusäo sanguínea cerebral / Disintegrative disorders of childhood: report of 2 clinical cases with cerebral blood perfusion study

Säo apresentados dois casos de síndrome de Heller (transtorno desintegrativo da infância), caracterizados por um desenvolvimento neuromotor e psicológico inicial normal até os sete anos de idade, seguidos por perda importante das habilidades cognitivas adquiridas, principalmente de comunicaçäo e de relacionamento social. E feita uma revisäo sobre aspectos clínicos e neurobiológicos dessa síndrome. Verificou-se uma sobreposiçäo curiosa entre os sintomas de nossas pacientes com síndrome de Heller e sintomas característicos da síndrome de Kluver-Buck. No Spect, uma das pacientes apresentou hiperperfusäo frontal e a outra, hipoperfusäo temporal esquerda. Estudos com maiores casuísticas, grupos-controle e métodos de Neuroimagem estrutural e funcional deveräo ser conduzidos para esclarecer a fisiopatologia da síndrome de Heller e o possível envolvimento das estruturas mesolímbicas.

Post-encephalitic Kluver-Bucy syndrome.

Kluver-Bucy syndrome is a rare amalgamation of neurobehavioural signs and symptoms seen infrequently in humans following insult to bilateral temporal lobes. This report presents a case which along with emerging symptoms of Kluver-Bucy syndrome, developed Attention-Deficit/Hyperactivity Disorder (ADHD) consequent to viral encephalitis. The case highlights the presentation and management of this syndrome in a six-year-old girl with primary focus on the implications of Kluver-Bucy symptoms in current clinical practice involving neurobehavioural syndromes in children.

A case of Pick's disease presenting with progressive nonfluent speech

BACKGROUND AND SIGNIFICANCE: Frontotemporal dementia is a behavioral disorder arising from nonAlzheimer's disease atrophy of frontal and anterior temporal lobe. Clinical manifestations include frontal lobe dysfunction. Kluver-Bucy syndrome or progressive language impairments. Two types of histological change underline, the atrophy. The commoner pathology is nerve cell loss and spongiform change with astrocytic gliosis. The second one is typical Pick-type histology characterized by intraneuronal inclusion body and astrocytic gliosis. We report a case with biopsy proved Pick's disease presenting with progressive nonfluent speech. CASE: A 41-years, old right-handed woman developed progressive language impairment over a period of 6 months. Brain MRI revealed asymmetric frontotemporal cortical atrophy more severe on the left side and 18F-FDG-brain, PET showed hypometabolism in the same area. Neuropsychological test including Korean version-Western Aphasia Battery revealed non-fluent speech as well as frontal lobe, dysfunction. A biopsy from left frontal lobe, demonstrate neuronal loss and diffuse astrogliosis. In the cytoplasm of remaining neurons are eosinophilic inclusion bodies which are neurofilament-positive with immunostaining. Senile plaque, neurofibrillary tangle and cortical Lewy body were absent. COMMENT: We report a case presenting with progressive nonfluent speech whose imaging and pathological findings are compatible with Pick's disease, which may be the first biopsy proven case in Korea.