合并多器官功能衰竭的急性脑血管病患者神经内分泌的改变

本实验动态测定了急性脑血管病（ＡＣＶＤ）并发多器官功能衰竭（ＭＯＦ）患者血清生长激素（ＧＨ）、泌乳素（ＰＲＬ）、促黄体生长素（ＬＨ）、促滤泡成熟激素（ＦＳＨ）、睾酮（Ｔ）、孕酮（Ｐ）、雌二醇（Ｅ２）、皮质醇（Ｆ）和血浆促肾上腺皮质激素（ＡＣＴＨ）水平的变化，并与ＡＣＶＤ各疾病组对比。结果发现：ＧＨ、ＰＲＬ、ＡＣＴＨ、Ｆ及ＦＳＨ水平显著升高；ＡＣＶＤ并发ＭＯＦ重型患者（ＭＯＦ积分＞４分）ＰＲＬ、ＧＨ、Ｆ水平显著高于轻型患者（ＭＯＦ积分≤４分）。结果提示ＰＲＬ、ＧＨ和Ｆ可能参与了ＡＣＶＤ并ＭＯＦ的病理生理过程     

垂体多激素腺瘤的临床病理分析及免疫电镜观察

应用免疫组化及免疫电镜技术，对３０例垂体多激素腺瘤患者进行了观察。结果显示：二种激素阳性者１８例，包括生长激素－催乳素（ＧＨ－ＰＲＬ）腺瘤１６例、生长激素－促甲状腺激素（ＧＨ－ＴＳＨ）腺瘤１例、生长激素－促肾上限皮质激素（ＧＨ－ＡＣＴＨ）腺瘤１例；三种激素阳性３例，包括生长激素－催乳素－促甲状腺激素（ＧＨ－ＰＲＬ－ＴＳＨ）腺瘤１例、生长激素－黄体生成素－卵泡刺激素（ＧＨ－ＬＨ－ＦＳＨ）腺瘤２例；四种激素阳性９例，包括生长激素－催乳素－黄体生成素－卵泡刺激素（ＧＨ－ＰＲＬ－ＬＨ－ＦＳＨ）腺瘤１例、生长激素－促甲状腺激素－黄体生成素－卵泡刺激素（ＧＨ－ＴＳＨ－ＬＨ－ＦＳＨ）腺瘤８例。免疫电镜观察到一个瘤细胞内可含一种激素，也可含多种激素，支持垂体多激素腺瘤既有单细胞起源，又有多细胞起源的观点。对垂体多激素腺瘤的临床病理特点、诊断及鉴别诊断进行了讨论。     

重症肌无力患者血清sIL—2R的研究

目的 探讨重症肌无力（ＭＧ）患者血清可溶性ＩＬ－２Ｒ（ｓＩＬ－２Ｒ）水平的变化及临床意义。方法 应用单克隆抗体，采用免疫酶标ＥＬＩＳＡ法检测了３８例不同临床类型（Ｉ，ⅡＡ，ⅡＢ型）ＭＧ患者和３５例健康对照者的血清ＳＩＬ－２Ｒ水平。结果 ＭＧ患者血清ｓＩＬ－２Ｒ水平显著高于健康对照组（Ｐ〈０．０１），伴有胸腺瘤和病情较严重的ＭＧ患者血清ｓＩＬ－２Ｒ水平的增高也较显著（Ｐ〈０．０５）。结论 ＭＧ患者血     

急性脑外伤患者垂体－性腺轴的功能变化（论著摘要）

急性脑外伤患者垂体－性腺轴的功能变化（论著摘要）元小冬，许亚茹，梁友平，米振洲（唐山开滦矿务局总医院神经科，唐山０６３０００）测定５７例急性脑外伤和３１例正常对照者的血清催乳素（ＰＲＬ）、卵泡刺激素（ＦＳＨ）、促黄体生成素（ＬＨ）、雌二醇（Ｅ２）、孕...     

男性肝豆状核变性下丘脑-垂体-睾丸轴功能研究

目的了解男性肝豆状核变性（ＷＤ）病人下丘脑－垂体－睾丸轴激素分泌功能。方法用放射免疫法测定２６例病人血清垂体、性激素水平。结果孕酮（Ｐ）显著低于正常对照组（Ｐ＜０．０５），生长激素（ＧＨ）、黄体生成素（ＬＨ）、雌二醇（Ｅ２）亦显著降低（Ｐ＜０．０１），性激素结合球蛋白（ＳＨＢＧ）则显著高于正常对照组（Ｐ＜０．００１），２０例患者进行促性腺激素释放激素（ＧｎＲＨ）试验，发现腺垂体对外源性ＧｎＲＨ的刺激反应迟钝。结论ＷＤ垂体、性腺细胞中部分酶的活性降低，使二者激素分泌功能降低；同时肝的铜沉积扰乱了蛋白代谢，使ＳＨＢＧ升高，进一步降低了性激素的生物效应     

精神分裂症患者的性激素分泌变异

为研究精神分裂症患者垂体促性腺激素及外周性激素的功能状态，探讨性激素水平改变与性有关症状和药物治疗的关系，采用放射免疫法测定６０例（男４０例，女２０例）精神分裂症患者的血清促卵泡激素（ＦＳＨ）、黄体生成素（ＬＨ）、催乳素（ＰＲＬ）、睾丸酮（Ｔ）、雌二醇（Ｅ２）等激素水平，并与２０名（男女各１０名）正常人对照。结果显示，试验组中男性ＬＨ和女性ＰＲＬ水平明显低于对照组，女性患者的Ｔ与Ｅ２水平显著升高。按性有关症状的有无分为两组，两组间性激素水平无显著性差异。药物治疗前后男患者ＰＲＬ，女患者ＦＳＨ、ＰＲＬ、Ｔ和Ｅ２等激素分泌改变非常显著。氯丙嗪、舒必利可致明显的高ＰＲＬ血症，但氯氮平无明显影响。提示精神分裂症患者性腺轴存在功能失调。性有关症状与性激素水平改变无关。抗精神病药治疗可导致性激素分泌紊乱。     

绝经后妇女脑梗死患者性激素变化与胰岛素抵抗的关系

目的：为探讨绝经后妇女脑梗死患者性激素水平变化及与胰岛素抵抗的关系。方法：对３３例绝经１年以上妇女脑梗死患者（Ａ组）与２１例绝经１年以上无脑梗死健康妇女（Ｂ组）的血清雌二醇（Ｅ２）、促卵泡刺激素（ＦＳＨ）、促黄体生成素（ＬＨ）、睾酮（Ｔ）、血浆葡萄糖（Ｇ）、血浆胰岛素（ＩＮＳ）及胰岛素敏感指数（ＩＳＩ）进行检测。结果：Ａ组Ｅ２水平较Ｂ组明显降低,ＦＳＨ、ＬＨ、Ｔ水平显著增高。相关分析发现,Ｅ２与ＩＳＮ是显著负相关,而Ｅ２与ＩＳＩ呈显著正相关;Ｔ与ＩＮＳ呈显著正相关,与ＩＳＩ是显著负相关。结论：绝经后妇女脑梗死患者存在严重的性激素失调,且与胰岛素抵抗并存,可能参与绝经后妇女脑梗死发生和发展。     

女性肝豆状核变性下丘脑-垂体-卵巢轴功能研究

目的 研究女性肝豆状核变性（ＨＬＤ）患者下丘脑－垂体－卵巢轴激素分泌功能。方法 放免法测定１３例ＨＬＤ患者（ＨＬＤ组）和１５名正常对照组血清垂体、性激素水平。结果 ＨＬＤ患者卵泡刺激素（ＦＳＨ）显著低于正常组（Ｐ〈０．０５）,孕酮（Ｐ）、黄体生成素（ＬＨ）、雌二醇（Ｅ２）亦显著降低（Ｐ〈０．０１）,性激素结合球蛋白（ＳＨＢＧ）则显著高于正常对照组（Ｐ〈０．００１）,１０例患者行促性激素释放激素（ＧｎＲＨ）试验,发现患者腺垂体对外源性ＧｎＲＨ的刺激反应迟钝。结论 ＨＬＤ患者垂体、性腺细胞中部分酶的活性降低,使激素分泌功能降低;同时肝的铜沉积扰乱了蛋白代谢,使ＳＨＢＧ升高,进一步降低了性激素的生物效应。     

重症肌无力病人血清中IgM型乙酰胆碱受体,突触前膜抗体检测及 …

应用ＡＢＣ－ＥＬＩＳＡ法检测了９６例重症肌无力（ＭＧ）病人血清中ＩｇＭ－ＡｃｈＲ、Ｐｓｍａｂ。发现ＭＧ组中，有１８例（１８．７％）ＩｇＭ－ＡｃｈＲａｂ阳性；１０例（１０．４％）ＩｇＭ－Ｐｓｍａｂ阳性。ＩｇＭ－Ｐｓｍａｂ阳性频见于病程〈２年的ＭＧ病人；而与ＭＧ组病人的年龄和临床分型均无显著的相关性（Ｐ〉０．０５）。结果提示ＩｇＭ型抗体也与ＩｇＭ型抗体共同参与了ＭＧ的发病机制，且提示有神经肌肉接头突触     

垂体腺瘤细胞体外培养激素测定

对３０例垂体腺瘤细胞的培养液进行了催乳素（ＰＲＬ）、生长激素（ＧＨ）、促甲状腺激素（ＴＳＨ）、黄体生成激素（ＬＨ）和促卵泡激素（ＦＳＨ）含量的放射免疫或免疫放射测定，发现经临床及病理确定的功能性腺瘤在体外培养过程中均能分泌高水平的相应激素，８／１０例无功能腺瘤亦可分泌ＬＨ、ＦＳＨ和ＰＲＬ，但其激素分泌量明显低于泌乳素和促性腺素腺瘤的分泌量。依据测定结果对垂体腺瘤组织进行初步功能分类，并对体内外激素     

Regional and cellular localisation of GABA(A) receptor subunits in the human basal ganglia: An autoradiographic and immunohistochemical study.

The regional and cellular localisation of gamma-aminobutyric acid(A) (GABA(A)) receptors was investigated in the human basal ganglia using receptor autoradiography and immunohistochemical staining for five GABA(A) receptor subunits (alpha(1), alpha(2), alpha(3), beta(2, 3), and gamma(2)) and other neurochemical markers. The results demonstrated that GABA(A) receptors in the striatum showed considerable subunit heterogeneity in their regional distribution and cellular localisation. High densities of GABA(A) receptors in the striosome compartment contained the alpha(2), alpha(3), beta(2, 3), and gamma(2) subunits, and lower densities of receptors in the matrix compartment contained the alpha(1), alpha(2), alpha(3), beta(2,3), and gamma(2) subunits. Also, six different types of neurons were identified in the striatum on the basis of GABA(A) receptor subunit configuration, cellular and dendritic morphology, and chemical neuroanatomy. Three types of alpha(1) subunit immunoreactive neurons were identified: type 1, the most numerous (60%), were medium-sized aspiny neurons that were immunoreactive for parvalbumin and alpha(1), beta(2,3), and gamma(2) subunits; type 2 (38%) were medium-sized to large aspiny neurons immunoreactive for calretinin and alpha(1), alpha(3), beta(2,3), and gamma(2) subunits; and type 3 (2%) were large sparsely spiny neurons immunoreactive for alpha(1), alpha(3), beta(2,3), and gamma(2) subunits. Type 4 neurons were calbindin-positive and immunoreactive for alpha(2), alpha(3), beta(2,3), and gamma(2) subunits. The remaining neurons were immunoreactive for choline acetyltransferase (ChAT) and alpha(3) subunit (type 5) or were neuropeptide Y-positive with no GABA(A) receptor subunit immunoreactivity (type 6). The globus pallidus contained three types of neurons: types 1 and 2 were large neurons and were immunoreactive for alpha(1), alpha(3), beta(2,3), and gamma(2) subunits and for parvalbumin alone (type 1) or for both parvalbumin and calretinin (type 2); type 3 neurons were medium-sized and immunoreactive for calretinin and alpha(1), beta(2, 3), and gamma(2) subunits. These results show that the subunit composition of GABA(A) receptors displays considerable regional and cellular variation in the human striatum but are more homogeneous in the globus pallidus.     

Neurologic disease in biopsy-proven giant cell (temporal) arteritis

Neurologic findings were studied in 166 consecutive patients with biopsy-proven giant cell (temporal) arteritis. Neurologic problems occurred in 51 patients (31%): neuropathies (23), TIA/strokes (12), neuro-otologic syndromes (11), tremor (6), neuropsychiatric syndromes (5), tongue numbness (3), and myelopathy (1). Neuro-ophthalmologic problems occurred in 35 patients (21%): amaurosis fugax (AF) (17), permanent vision loss (PVL) (14), scintillating scotoma (8), and diplopia (3). Abnormalities in large arteries in 52 patients (31%) included bruits and diminished pulses. The carotid artery was involved in 31 patients (bilateral in 58%). Overall, 35% of patients with carotid disease had TIA/stroke, AF, or PVL.     

Bilateral involvement of a single cranial nerve: analysis of 578 cases

The author reviewed 34 years of personal experience with inpatients in a large municipal hospital to analyze the seats and causes of involvement of single pairs of cranial nerves. Among 578 cases, the sixth (n = 234) and second (211) nerves predominated, followed by the fourth (48), seventh (30), third (27), and eighth (18) cranial nerves. Trauma (99), infection (94), tumor (92), increased intracranial pressure (85), vascular disease (74), and demyelination (66) were common causes.     

Hepatocyte growth factor in cerebrospinal fluid in neurologic disease

OBJECTIVE: To investigate hepatocyte growth factor (HGF) concentration in cerebrospinal fluid (CSF) in neurologic disease. MATERIALS AND METHODS: We determined CSF concentration of HGF with human-HGF-specific enzyme-linked immunosorbent assays (ELISA) in 121 patients: Alzheimer's disease (AD) (33), amyotrophic lateral sclerosis (ALS) (10), Parkinson's disease (PD) (5), progressive supranuclear palsy (PSP) (3), spinocerebellar degeneration (7), acute disseminating encephalomyelitis (ADEM) (6), human T-lymphotropic virus-1 (HTLV-1)-associated myelopathy (HAM) (6), multiple sclerosis (MS) (7), aseptic meningitis (AM) (12), and peripheral neuropathy and myopathy as control diseases (32). RESULTS: HGF concentrations in CSF were significantly higher with diseases of the central nervous system (CNS) than control diseases and were slightly higher with AD than other neurodegenerative diseases. Values were highest with ADEM but decreased during corticosteroid treatment. We found no relationship between HGF in CSF and CSF cells or protein, immunoglobulin index, or Q albumin. CONCLUSION: It is suggested that high concentrations of HGF in CSF may be partially related to CNS pathology, especially to demyelinating disease.     

慢性肾血管性高血压大鼠脑细胞膜磷脂及游离脂肪酸含量变化

采用SD大鼠慢性肾血管性高血压模型，高效液相和气－液色诣定量法测定脑细胞胺磷脂、游离脂肪酸（FreeFattyAcidFFA）组分变化．结果显示：1．高血压组脑细胞膜磷脂组分中PI显著低于对照组（P＜0．O5），PS、PE、PC，组分轻微下降（P＞0．05）．2．FFA组分中C20；4，C22；6水平显著高于对照组（P＜0.01，P＜0．O5），C18：0水平显著低于对照组（P＜0．05），C16：0和C18：1轻度降低（P＞0．05）．提示继发性高血压大鼠伴有脑细胞膜磷脂、FFA代谢障碍．     

血液透析患者透析间期颅内动脉血流动力学变化的临床研究

的   探讨血液透析患者透析间期颅内动脉血流动力学动态变化特征。 方法  本研究采用病例对照研究方法。以经颅多普勒超声（transcranial duplex sonography,TCD）对30例血液透析患者透析间期大脑前动脉（anterior cerebral artery,ACA）、大脑中动脉（middle cerebral artery,MCA）、大脑后动脉（posterior cerebral artery,PCA）以及基底动脉（basilar artery,BA）血流动力学变化进行评估,检测双侧动脉平均流速（mean velocity,MV）、阻力指数（resistant index,RI）及搏动指数（pulsatile index,PI）;以肾功能正常、年龄、性别匹配的28例门诊TCD检查患者为对照组。分析两组间差异并采用Logistic回归方法分析颅内动脉血流动力学改变的相关风险因素。 结果  血液透析组ACA、MCA、PCA、BA的MV分别为（67.3±12.7）cm/s（P＝0.03）、（76.5±13.4）cm/s（P＝0.04）、（66.7±12.5）cm/s（P＝0.04）及（51.3±10.7）cm/s（P＝0.03）,与对照组比较均增加;血液透析组RI、PI与对照组差异无显著性。多因素Logistic回归结果显示,血液透析患者ACA、MCA、PCA、BA的MV增加与贫血相关,其比值比（odds ratio,OR）及95%可信区间（confidence interval,CI）分别为：1.2（1.1～2.9）、2.2（1.8～3.6）、1.9（1.7～3.2）和1.6（1.5～3.1）;上述颅内动脉MV亦与高血压相关,OR值及95%CI分别为：2.4（1.9～3.9）、2.9（1.7～4.2）、2.1（1.7～3.9）和2.6（1.8～3.1）。 结论  血液透析患者透析间期颅内动脉平均血流速度增加,此改变与贫血及高血压相关。     

Autonomic impairment in painful neuropathy

OBJECTIVES: 1) To determine the degree and distribution and quantitate the severity of autonomic impairment in painful neuropathy (PN). 2) To assess the role of autonomic testing in evaluating PN. METHODS: The authors studied 92 patients with PN (60 women and 32 men, age 56.9 +/- 12.4 years) using: 1) autonomic reflex testing (ART), Quantitative Sudomotor Axon Reflex Test (QSART), cardiac-vagal, head-up tilt, and surface skin temperature; 2) autonomic symptoms questionnaire; 3) nerve conduction (NCS) and laboratory studies; 4) quantitative sensory testing; 5) skin biopsy; and 6) Composite Autonomic Symptoms Score (CASS) scale to grade ART results from 0 (normal) to 10 (autonomic failure). RESULTS: Autonomic involvement in PN had characteristic features. Main symptoms were pain, secretory and skin vasomotor signs, hypertension, and impotence. ART results were abnormal in 86 (93.5%) (CASS < 4), QSART in 67 (72.8%), cardiac-vagal index in 58 (63%), skin temperature in 51 (55.4%), orthostatic hypertension in 39 (42.3%), and family history of PN in 26 (21%) of patients. Group 1 (abnormal NCS) (n = 45) had more severe ART and sensory abnormalities than the Group 2 (normal NCS) (n = 47): 1) CASS 2.0 +/- 0.96 vs 1.55 +/- 0.88 (p < 0.01), cardiac-vagal index (p < 0.02), skin temperature (p < 0.02), hypertension (p < 0.03), cooling (p < 0.002), and vibration (p < 0.0005) thresholds. CONCLUSIONS: Autonomic symptoms in painful neuropathy are predominantly cholinergic and form a unique constellation of features that are distinct from other autonomic neuropathies.     

Basic and escalating immunomodulatory treatments in multiple sclerosis: Current therapeutic recommendations

Abstract   This review updates and extends earlier Consensus Reports related to current basic and escalating immunomodulatory treatments in multiple sclerosis (MS). The recent literature has been extracted for new evidence from randomized controlled trials, open treatment studies and reported expert opinion, both in original articles and reviews, and evaluates indications and safety issues based on published data. After data extraction from published full length publications and critically weighing the evidence and potential impact of the data, the review has been drafted and circulated within the National MS Societies and the European MS Platform to reach consensus within a very large group of European experts, combining evidence-based criteria and expert opinion where evidence is still incomplete. The review also outlines a few areas of controversy and delineates the need for future research. Appendix    Multiple Sclerosis Therapy Consensus Group (MSTCG) Austria. U. Baumhackl (St. P?lten), T. Berger (Innsbruck), F. Deisenhammer (Innsbruck), F. Fazekas (Graz), M. Freimüller (Hermagor), H. Kollegger (Vienna), W. Kristoferitsch (Vienna), H. Lassmann (Vienna), H. Markut (V?cklabruck), S. Strasser-Fuchs (Graz), K. Vass (Vienna). Belgium. C. Sindic (Brussels), M.B. D’hooghe (Melsbroek) Croatia. S. Podobnik (Sarkanji) Czech Republic. E. Havrdova (Prague) Finland. I. Elovaara (Tampere) France. M. Clanet (Toulouse), C. Confavreux (Lyon), G. Edan (Rennes), C, Lubetzki (Paris) Germany. H. Altenkirch (Berlin), S. Bamborschke (Bernau), K. Baum (Hennigsdorf), A. Bayas (Augsburg), R. Benecke (Rostock), W. Brück (G?ttingen), M. Buttmann (Würzburg), A. Chan (Bochum), M. Daumer (Munich), D. Dommasch (Bielefeld), W.G. Elias (Hamburg), E. Fasshauer (Halle), P. Flachenecker (Bad Wildbad), R. Gold (Bochum), J. Haas (Berlin), G. Haferkamp (Hanover), P. Haller (Osnabrück), H.-P. Hartung (Düsseldorf), C.Heesen (Hamburg), M. Heibel (Hachen), F. Heidenreich (Hannover), B. Hemmer (München), T. Henze (Niddenau), R. Hohlfeld (Munich), R. W. C. Janzen (Frankfurt/Main), G. Japp (K?nigstein), S. Jung (Dillingen), E. Jügelt (Sundern/Hachen), B. Kallmann (Bamberg), B. C. Kieseier (Düsseldorf), C. Kleinschnitz (Würzburg), J. K?hler (Hamburg), W. K?hler (Wermsdorf), W. K?lmel (Erfurt), N. K?nig (Berg), G. Lehrieder (Bad Windsheim), V. Leussink (Düsseldorf), K. Lowitzsch (Ludwigshafen), M. M?urer (Erlangen), U. Manegold (G?ttingen), A. Melms (Tübingen), J. Mertin (Amtzell), S.G. Meuth (Würzburg), O. Neuhaus (Sigmaringen), P. Oschmann (Bayreuth), H.-F. Petereit (Cologne), M. Pette (Dresden), D. P?hlau (Asbach), D. Pohl (Paris), P. Rieckmann (Würzburg/Vancouver), K. Ruprecht (Homburg), M. Sailer (Magdeburg), H. Schipper (Bernburg), S. Schmidt (Bonn), G. Schock (Gera), M. Schulz (Ueckermünde), S. Schwarz (Mannheim), G. Schwendemann (Bremen), D. Seidel (Isselburg), N. Sommer (Marburg/G?ppingen), M. Stangel (Hannover), E. Stark (Offenbach), A. Steinbrecher (Regensburg), G. Stoll (Würzburg), K. V. Toyka (Würzburg), H. Tumani (Ulm), R. Voltz (Cologne), F. Weber (Munich), F. Weilbach (Bad Kissingen), H. Wiendl (Würzburg), H. Wieth?lter (Stuttgart), B. Wildemann (Heidelberg), U. K. Zettl (Rostock), T. Ziemssen (Dresden), F. Zipp (Berlin), R. Zschenderlein (Berlin). Greece. A. Orologas (Thessaloniki) Hungary. A. Guseo (Székesfehérvár) Iceland. S. Bergmann (Reykjavik) Italy. M. Pugliatti (Sassari) Norway. K.-M. Myhr (Bergen) Spain. G. Izquierdo (Sevilla), Switzerland. C. Bassetti (Zurich), K. Beer (St. Gallen), S. Beer (Valens), U. Buettner (Aarau), M. Chofflon (Geneva), A. Gass (Basel/Mannheim), N. Goebels (Zurich), M. G?tschi-Fuchs (Knoblisbühl), L. Kappos (Basel), J. Kesselring (Valens), H.-P. Ludin (St. Gallen), H. Mattle (Bern), M. Schluep (Lausanne), C. Vaney (Montana-Vermala). U.K. D. Bates (Newcastle u. Tyne)     

Three dimensional analysis of retinal neuropeptides and amine in the chick

Three dimensional analysis of retinal neuropeptides and monoamine-containing amacrine cells were performed on flat-mount preparations of the chick retina by using indirect immunofluorescence method. somatostatin (SOM), neurotensin (NT), leu-enkephalin (ENK), vasoactive intestinal polypeptide (VIP), substance P (SP), corticotropin releasing factor (CRF), avian pancreatic polypeptide (APP), glucagon (GLC), 5-hydroxytryptamine (5HT) and tyrosine hydroxylase (TH) were examined with specific antisera. To localize these substances in the amacrine cells, and to see in which layers their processes arborize, frozen sections were examined. There were four patterns of distribution. (1) Substances with more immunoreactive cells in the central than in the peripheral portions (SOM, NT, VIP, SP, GLC, 5HT), (2) Substances with more immunoreactive cells in the peripheral portion than in the central portion (APP), (3) Substances for which such cells were evenly distributed (TH), and (4) Substances with more immunoreactive cells in the inferior than in the superior portion (CRF). Subtypes were identified among the amacrine cells containing single peptides or monoamine.     

Epilepsy in severe motor and intellectual disabilities syndrome (SMIDS)--a clinical and electroencephalographic study of epileptic syndromes

The subjects were 106 SMIDS with epilepsy. They were classified into four epileptic syndromes: (1) SE-MISF (34.0%), (2) SGE (25.5%), (3) SLRE (20.7%), and (4) epileptic discharge-free patients (EDFP) (19.8%). Clinical electroencephalographic studies elucidated the following result: (1) The seizure disappearance rate was the highest in SLRE (54.5%), and it decreased in the order of EDFP (47.6%), SE-MISF (36.1%), and SGE (11.1%). (2) Status epilepticus was most frequently seen in SGE (62.4%), but it was not so often seen in EDFP (14.3%) or SLRE (22.7%). (3) The age at seizures onset was the lowest in SE-MISF (0.84 years), and it increased in the order of SLRE (1.3), SGE (2.3), and EDFP (6.7). (4) The rate of Ohshima's classification 1 was highest in SE-MISF (61.1%) and lowest in SGE (40.7%). In conclusion, epileptic syndrome and EEG findings are good indicators for predicting the seizure prognosis and some of the clinical features, and the majority of epileptic syndromes could be classified by the very first EEG findings. Since epilepsy in SMIDS is so frequent (70.3%) and intractable (seizure disappearance rate more than 3 years, 36.2%), more attention should be paid to electroencephalography and epileptic seizures in SMIDS.