Neurocysticercosis presenting as Weber's syndrome

This case report describes a rare, non-epileptic manifestation of neurocysticercosis where a 22-year-old male presented with acute onset right 3rd nerve palsy with left hemiplegia (Weber syndrome). Computerized tomography and magnetic resonance imaging revealed cysticercus granuloma. The patient improved and became asymptomatic with steroid treatment. Recognizing this clinical entity would avoid unnecessary antituberculous treatment and surgical intervention.     

Absence of maternal A3243G mtDNA mutation and reversible hyperglycemia in a patient with MELAS syndrome

We report the unusual features of a female patient who had MELAS-specific A3243G mutation in mitochondrial DNA (mtDNA) and diabetes mellitus (DM). The patient showed mitochondrial myopathy, encephalopathy, lactic acidosis, and deafness but lacked the stroke-like episode. Acute hyperglycemia was noted after one attack of status epilepticus. Molecular genetic analysis demonstrated a heteroplasmic A3243G point mutation in the mtDNAs of muscle, blood cells and hair follicles. Glucagon stimulation test exhibited marked depression of pancreatic beta-cell function. However, in a further study neither this mutation, nor MELAS syndrome or DM, was found in all of her maternal relatives. A series of follow-up studies for beta-cell function also showed gradual improvement. The pedigree study led us to believe that this A3243G mutation arose from the germ line cells or occurred later in somatic tissues of the patient. We also suggest that the A3243G mutation of mtDNA may elicit the pathogenesis of a subtype of DM. Nevertheless, environmental stress may be another important factor for provocation of the disease.     

Posterior reversible encephalopathy syndrome in a patient following binge liquorice ingestion

Journal of Neurology -     

HELLP syndrome with reversible posterior leukoencephalopathy

We report the case of a 30-year-old primipara who developed a triplet pregnancy after having received artificial insemination. At the end of the eighth month of pregnancy she had a generalized tonic-clonic seizure and at the same time blood chemistry was indicative of HELLP syndrome. Brain MRI and EEG were altered immediately after the episode but returned to normal during a 6-month follow-up period. Received: 15 December 2002 / Accepted in revised form: 14 April 2003 Correspondence to: E. Marano     

Ictal asystole in a patient with posterior reversible encephalopathy syndrome (PRES) and seizures

We describe a case of ictal asystole in a patient with posterior reversible encephalopathy syndrome (PRES), which has never been described previously. Ictal asystole is rare and has possible serious medical consequences, including syncope, and may be a potential mechanism for sudden unexpected death in epilepsy (SUDEP). Awareness that PRES may be observed with recurrent ictal asystole may aid in the recognition and treatment of seizures in this condition and the prevention of asystole‐associated complications.     

Posterior reversible encephalopathy syndrome induced by intracranial hypotension in a postpartum patient

Abstract

Objective: A number of hormonal, physiologic, immunologic, and hemodynamic changes can cause a series of central nervous system-related problems in pregnant and postpartum women. Posterior reversible encephalopathy syndrome (PRES) is commonly seen in these conditions. However, PRES during pregnancy and the postpartum period are not always due to pregnancy.

Methods: We describe a patient who presented with headache followed by an epileptic seizure after cesarean section and whose computed tomography (CT) showed bilateral low-density lesions in the frontal lobe. To explore the pathogenesis, we further examined the patient with brain magnetic resonance imaging (MRI) and lumbar puncture.

Results: Brain MRI revealed vasogenic edema in the frontal lobe and temporal-occipital regions of both hemispheres. MRI of the brain with contrast showed diffuse enhancement of the supratentorial dura mater and decreased of bilateral lateral ventricles. There was no abnormality in brain magnetic resonance angiography and magnetic resonance venography. Bloody cerebrospinal fluid flowed very slowly during lumbar puncture.

Conclusion: These findings suggest that, although rare, intracranial hypotension in postpartum patients may be a cause of PRES.     

A case of neuroleptic malignant syndrome associated with reversible leukoencephalopathy]

We report a case of neuroleptic malignant syndrome associated with reversible leukoencephalopathy. The patient was a 60-year-old woman. Soon after ingesting an antipsychotic drug, the patient developed neuroleptic symptoms. After hydration and dantrolene sodium were administered, muscular rigidity gradually improved and serum levels of CK became normal. On the 7th hospital day, however, she fell into coma and showed tetraplegia. Although brain CT was normal on admission, diffuse low density areas were observed on the parieto-occipital cerebral white matter. The same lesions were observed on T2-weighted MRI. On the 20th hospital day, after giving her glycerol and adrenocorticosteroid, not only abnormal neurological findings but also abnormal CT and MRI findings disappeared. There were only two case reports of leukoencephalopathy with malignant syndrome in the literature. This is a rare case of reversible leukoencephalopathy with neuroleptic malignant syndrome due to the antipsychotic drug.     

可逆性后部白质脑病综合征合并HELLP综合征的临床分析

目的分析可逆性后部白质脑病综合征(PRIS)合并HELLP综合征患者的临床特征,提高对该病的认识。方法回顾性分析我院诊治1例及国内文献报道4例.PRLS合并HELLP综合征的临床表现、实验室及影像学检查、治疗和转归。结果5例患者中出现严重高血压4例,头痛5例,癫痫发作5例,视觉异常5例,意识障碍5例,精神障碍2例,局灶性神经系统定位体征3例,肢体水肿1例。实验室检查均提示有溶血、肝酶升高、血小板减少。5例患者头部cT或MRI检查均显示双侧顶枕叶白质为主的对称性异常,部分累及额叶、颞叶,病灶在cT为低密度灶,MRI为长T_1长T_2信号灶。结论 RPLS同时合并HELLP综合征是妊高征患者罕见的并发症,充分认识其临床和影像学特点,有助于及时诊断和治疗。     

A pediatric case of reversible cerebral vasoconstriction syndrome with cortical subarachnoid hemorrhage

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare disorder characterized by acute onset, severe headache, with reversible vasoconstriction of cerebral arteries often accompanied by additional neurological symptoms. This syndrome is seen mainly in middle-aged adults, predominantly women. Herein, we report on a pediatric case of RCVS with cortical subarachnoid hemorrhage (SAH). A 12-year-old boy developed acute, severe headache with paralysis of lower extremities causing gait disturbance after administration of eletriptan. Brain magnetic resonance angiography (MRA) revealed multifocal narrowing of the cerebral arteries, whereas magnetic resonance imaging (MRI) demonstrated sulcal hyperintensity on fluid-attenuated inversion recovery, consistent with cortical SAH. The patient's clinical symptoms resolved spontaneously after a few days and the MRI and MRA findings disappeared 3months later, suggesting a diagnosis of RCVS. Eletriptan might cause vasoconstriction of cerebral arteries. Although most patients with RCVS are adults and pediatric cases are rare, RCVS should be considered in a child complaining of severe headache.