Hypoparathyroidism: A rare treatable cause of epilepsy – report of two cases

Hypoparathyroidism occurs due to insufficient production of parathyroid hormone to maintain extracellular calcium levels within the normal range. The acute clinical symptoms and signs of hypoparathyroidism are those of hypocalcaemia, ranging from tingling and numbness of limb extremities to intractable seizures. Often seizures are mistaken for epilepsy. Though hypoparathyroidism is not uncommon, the diagnosis is often missed due to its unusual clinical manifestation. This is the first documented report with vitamin D, Parathormone levels and urinary biochemical parameters from India. We present two cases of hypoparathyroidism who presented with seizures along with a short review of literature.     

Hemimegalencephaly: clinical implications and surgical treatment

Introduction Hemimegalencephaly (HME) is a quite rare malformation of the cortical development arising from an abnormal proliferation of anomalous neuronal and glial cells that generally leads to the hypertrophy of the whole affected cerebral hemisphere. The pathogenesis of such a complex malformation is still unknown even though several hypotheses are reported in literature.Background HME can occur alone or associated with neurocutaneous disorders, such as neurofibromatosis, epidermal nevus syndrome, Ito’s hypomelanosis, and Klippel–Trenonay–Weber syndrome. The clinical picture is usually dominated by a severe and drug-resistant epilepsy. Other common findings are represented by macrocrania, mean/severe mental retardation, unilateral motor deficit, and hemianopia. The EEG shows different abnormal patterns, mainly characterized by suppression burst and/or hemihypsarrhythmia. Although neuroimaging and histologic investigations often show typical findings (enlarged hemisphere, malformed ventricular system, alteration of the normal gyration), the differential diagnosis with other disorders of the neuronal and glial proliferation may be difficult to obtain. Hemispherectomy/hemispherotomy is the most effective treatment to control seizure, and it also seems to provide good results on the psychomotor development when performed early, as demonstrated by the literature review and by the reported personal series reported here (20 children). The surgical therapy of HME, however, is still burdened by a quite high complication rate and mortality risk.     

Atypical presentation of NREM arousal parasomnia with repetitive episodes

The case report describes a distinct variant of non-REM (Rapid Eye Movement) arousal parasomnia, sleepwalking type, featuring repetitive abrupt arousals, mostly from slow-wave sleep, and various automatisms and semi-purposeful behaviours. The frequency of events and distribution throughout the night presented as a continuous status of parasomnia (' status parasomnicus '). The patient responded well to treatment typically administered for adult NREM parasomnias, and after careful review of the clinical presentation, objective findings and treatment outcome, sleep-related epilepsy was ruled out in favour of parasomnia.     

海人酸致大鼠海马结构GABA_B受体亚单位表达的研究

目的:探讨海人酸诱导大鼠颞叶癫(EP)发作后2种γ-氨基丁酸(GABA)受体亚单位GABABR亚单位1a(GBR1a)和GABABR亚单位2(GBR2)在EP发生、发展中的作用。方法:运用原位杂交及免疫组化法,检测EP发作后GABABR亚单位mRNA及蛋白在海马的表达。结果:致早期CA1和CA3区2种亚单位mRNA表达持续低下后逐渐增加,DG区则暂时性下降后很快回升;而免疫反应早期却未见明显改变,随后CA1和CA3区表达处于低水平,DG区和颞叶皮质表达下降后很快恢复。结论:致后2种GABAB受体亚单位基因和蛋白表达上调为颞叶EP的内源性自我保护机制。     

Evaluating therapeutic outcome in epileptic patients using the changes of interval and duration

BACKGROUND: Seizure frequency is in abnormal distribution, and it is not enough to express the trend of concentration using means, and its median loses a lot of information, thus it lacks of a standard for evaluating the therapeutic effects based on seizure frequency. OBJECTIVE: To establish a method for evaluating the therapeutic effects on anti-epileptic drugs using changes of interval and duration of seizure. DESIGN: A prospective cohort study. SETTING: Zhumadian Psychiatric Hospital. PARTICIPANTS: Outpatients and inpatients suffering from epilepsy attending firstly visited Zhumadian Psychiatric Hospital from June 2001 to June 2002 were enrolled. They were diagnosed as epileptic according to the International Classification of Epileptic Seizure by International League Against Epilepsy (1981) based on the clinical history, physical examination, and investigations. The interval time was no more than 6 months. Informed consent was obtained from all the subjects, and the study was approved by the hospital ethical committee. METHODS: ① For the first visit and each follow-up, the following data were recorded, including general demographic information, seizure type, the date and time of ictus, the duration of ictus, and inducement or situation related, according to which the following indexes could be calculated, including seizure styles, interval, duration, cluster frequency and cluster duration. The information from the first review was noted as annals A. The second interview was taken at the end of the evaluating period; the information from the second review was noted as annals B. The third interview was taken within two weeks after the second one; the information from the third review was noted as annals C. The annals B or the annals C were respectively compared with the annals A in the light of the same types or the same styles of the same patient. Summation of the scores of interval change and duration change of the same type or the same style and 5 of basic score was the score of a corresponding seizure type or a corresponding style of one patient. In order to test its reliability and validity, the score of change of frequency or duration plus 5 scores respectively was the score of frequency or duration. ② Reliability and validity were tested by calculating corresponding correlation coefficient with SPSS 11.0. ROC curve was used for developing diagnostic criterion of predicting therapeutic effects with SPSS 11.0. MAIN OUTCOME MEASURES: ① Reliability and validity; ② Diagnostic criterion for predicting therapeutic effects one year later. RESULTS: Totally 28 patients were involved in the final analysis of results. ① Reliability and validity were high: rinter-rater=0.98, rtest-retest=0.99, rconstruct validity=0.83. ② A total score > 6 was the optimal diagnostic criteria for predicting therapeutic effects one year later, in other words, a patient who scored more than 6 at the evaluating period may be seizure-free one year later. CONCLUSION: It is a potential tool for evaluating epileptic therapeutic outcome, and it can be diffusely used in interrelated fields after being further validated.     

中风后癫痫的临床研究

本文报告３６例中风后癫痫发作的临床和ＣＴ资料。通过回顾性病历复习和随访发现，中风后癫痫的发生率占同期住院中风患者的５．２６％，以蛛网膜下腔出血和脑栓塞发生癫痫比率最高，分别为１５％和１２．５％。癫痫发作与ＣＴ所见病灶分布密切相关，皮质病灶较皮质下病灶更易发生病病。癫痫发作可发生于中风后任何时期，但早期癫痫以出血性中风多见，而迟发性癫痫则更多见于脑梗塞患者。     

Adrenocorticotropic Hormone Immunoreactivity in the Hippocampal Formation of Temporal Lobe Epilepsy Patients

Summary: Purpose: We wished to identify immunocytochemically the distribution of proopiomelanocortin-related peptides in the hippocampal formation of patients with epilepsy.
Methods: Surgical hippocampal specimens from temporal lobe epilepsy (TLE) patients and autopsy control tissue were examined immunocytochemically for ACTH, α-melanocyte-stimulating hormone (α-MSH) and α-endorphin.
Results: There was a dense distribution of ACTH-immunoreactive neurons in the hippocampal formation of patients with mesial TLE syndrome (MTLE). These hippocampal specimens showed significant cell loss. ACTH-positive neurons were most prominent in the subiculum, with scattered ACTH-immunoreactive neuronal elements distributed in the cornu ammonis fields and hilus. Light ACTH immunoreactivity was detected in the tumor-related epileptic hippocampal specimens, which showed minimal cell loss. Although autopsy control tissue from the hypothalamus showed intense ACTH staining patterns in cells and fibers, there was little or no ACTH immunoreactivity in the autopsy hippocampal tissue. The expression of ACTH immunoreactive elements was correlated with patterns of cell loss. No α-MSH- or β-endorphin-immunoreactive neurons were detected in any of the hippocampal specimens.
Conclusions: ACTH has anticonvulsant properties, and its novel expression in the glutamatergic subicular neurons, which provide the main outflow of the hippocampal formation, may represent an attempt by the damaged hippocampal circuit to restore the balance of excitatory/inhibitory neurotransmission in TLE.     

实验性激活黑质对大鼠癫痫发作时脑电图的影响

为探讨黑质抗癫痫作用的神经化学机制，实验用大鼠４０只，１２０万Ｕ青霉素（ｉｐ）诱发其癫痫（ＥＥＧ）发作．高波幅尖波连续发放型癫痫放电稳定时：（１）电刺激黑质（１０Ｈｚ、６Ｖ、０．２ｍｓ）即刻出现癫痫放电频率下降（Ｐ＜０．０５），连续刺激２５ｍｉｎ，效应最明显时停止电刺激，癫痫放电频率随即恢复，（２）黑质内微量注射多巴胺（ＤＡ）受体激动剂盐酸阿朴吗啡５μｇ，出现癫痫放电频率抑制现象（Ｐ＜０．０５），持续６０ｍｉｎ以上，部分动作癫痫放电消失后不再复现．（３）黑质内微量注射γ氨基丁酸（ＧＡＢＡ）４～５μｇ，明显易化大鼠癫痫放电频率（Ｐ＜０．０１），４０ｍｉｎ后癫痫放电频率大约是用药前的５倍，该效应可以被黑质内微量注射印防己毒素５μｇ阻断．结果提示：激活黑质ＤＡ系统功能活动有利于对抗青霉素致大鼠癫痫发作．     

苯妥英钠血清浓度监测（31例报告）

报告31例口服DPH治疗的门诊癫痫病人RIA法血清DPH浓度监测结果。在随访中记录的临床治疗反应。病人平均药物剂量为4．39±1．19mg/kg／d，不同病人服用同一剂量DPH，其血清药物旅度可相差9倍。22例获良好(较好)的治疗反应，其平均有效血清药物浓度为24．1±10．1μg／ml。9例出现中毒反应；血清药物浓度在25μg／ml以上时可出现眼球震颤、视物模糊，共济失调。DPH血清浓度监测有功于实现个体化给药方案，提高疗效并避免中毒。