以癫(癎)为首发症状的甲状旁腺功能减退症的临床表现和影像学特点

甲状旁腺功能减退症(hypoparathyroidism,HPT)是由于甲状旁腺素(parathyroidhormone,PTH)产生减少和(或)效应不足而引起的一组临床症候群,其特点为手足搐搦、癫(癎)样发作、低钙血症和高磷血症,以及典型的头部CT呈双侧基底节区对称性钙化等;临床多以神经症状为特点,部分以癫(癎)首发入院,容易误诊.     

以癫痫为首发症状的甲状旁腺功能减退症的临床表现和影像学特点

甲状旁腺功能减退症（hypoparathyroidism，HPT）是由于甲状旁腺素（parathyroidhormone，PTH）产生减少和（或）效应不足而引起的一组临床症候群，其特点为手足搐搦、癫疴样发作、低钙血症和高磷血症，以及典型的头部CT呈双侧基底节区对称性钙化等；临床多以神经症状为特点，部分以癫疴首发入院，容易误诊。此类患者极易产生颅内钙化，为了探讨实验室检查和影像学检查的诊断价值，笔者对我院2003—2006年收治的11例甲状旁腺功能减退症患者的临床资料进行回顾性分析。[第一段]     

甲状旁腺机能减退症与癫痫(附12例分析)

甲状旁腺机能减退症(甲旁减),常以神经精神症状为突出表现,现将我院近10年来收 治的以癫痫强直—痉挛性发作为主要表现的甲旁减分析如下: 本组12例中男7例,女5例。年龄16～37岁。均无甲状腺手术史。病史10天至7年,均经过抗癫痫治疗而未控制发作,本组12例均表现为强直—痉挛性癫痫发作,其中表现为癫痫持续状态10例,伴手足搐搦9例,有智能减退8例,佛斯特氏征阳性12例,陶瑟氏征阳性10例,双侧巴氏征阳性2例。脑电图异常8例,且在血钙正常后复查脑电图也恢复正常。头部CT有基底节区钙化者5例。血钙1.0～1.56mmol/L,磷2.86～3.     

表现为帕金森综合征的特发性甲状旁腺功能减退症一例

特发性甲状旁腺功能减退症(idiopathic hypoparathyroidism,IHP)是由原发性或获得性甲状旁腺激素(PTH)分泌不足引起机体钙磷代谢紊乱所导致的慢性代谢性疾病,其病因不明,临床表现多样,几乎可以累及任何器官。IHP发病率低,临床表现缺乏特异性,临床实践中易出现漏诊、误诊的现象。本文对1例确诊的类似帕金森综合征表现的IHP进行报道,期望通过该病例加深临床医生对IHP的认识。1病例报告患者男性,45岁,主因“四肢不自主抖动45年,加重5年”入院。患者于儿时无明显诱因出现双手抖动,后逐渐累及四肢,静止与运动时均可发作,紧张时症状明显。5年前患者抖动症状加重,表现为抖动幅度加大,未予诊治。1年前,患者因大幅度肢体抖动摔倒而就诊于当地医院,给予补钙治疗后症状缓解。     

甲状旁腺功能减退症5例脑CT诊断

甲状旁腺功能减退症是由于甲状旁腺PTH分泌不足或PTH对机体组织的作用丧失的一种状态 ,主要表现为低钙血症导致的四肢抽搐 ,精神神经改变和异位钙化 ,其中以脑基底神经节、大脑、小脑钙化为最常见。脑CT检查对该病诊断价值较高 ,现将我院遇到 5例结合文献对该病的脑CT诊断做初步分析。1　一般资料5例甲状旁腺功能减退症患者中 ,女 3例 ,男 2例 ,年龄 13～ 47岁 ,平均 3 0岁。临床上均有反复发作性四肢抽搐、无力 ,阵发性头痛伴全身酸痛。查体 :束臂加压试验及面神经叩击试验均呈阳性。生化检查均有低血钙、高血磷现象 (正常血清钙 :2 2…     

1例假性甲状旁腺功能减退报道

假性甲状旁腺功能减退( pseudohypoparathyroidism,PHP)是一种罕见的遗传病,其特点是终器官对甲状旁腺激素(parathyroid hormone,PTH)出现抵抗,表现为甲状旁腺功能减退的特征(低血钙、高血磷),但血清PTH高于正常,临床表现为感觉异常、肌肉痉挛、手足搐搦、喉及支气管痉挛、癫痫、锥体外系体征及基底节钙化等,常伴有Albright遗传性骨营养不良症(Albright's Hereditary Osteodystrophy,AHO),即身材矮小、肥胖、圆脸、指(趾)短小畸形、智力减退和异位骨化[1].现我们报道1例该病患者,以加强对本病的认识.     

以典型癫痫发作为临床表现的甲状旁腺机能减退症1例报道

正甲状旁腺机能减退症是由甲状旁腺激素分泌不足导致血钙下降、血磷升高,可对大脑、肌肉、骨骼、心脏、肾脏、胃肠道等多系统造成损害,其中2/3的患者可能合并癫痫发作。由于其临床表现复杂多样,患者常因某一系统或器官的症状更加突出而就诊于其他专科,从而误诊。国内数据显示误诊最长时间长达14年,误诊疾病居于首位的为癫痫(65.79%)。误诊为癫痫意味着患者需要长时间的口服抗癫痫药物,并增加患者及整个家庭的经济及精神负担。正确认识本病对诊断、治疗、预后     

特发性甲状旁腺机能减退与癫痫发作

报道６例以癫痫为主人表现的特发性甲状旁腺机能减退患者，男４例，女２例，年龄２３－３７岁，平均２８．３岁。６例均为强直一阵挛发作，其中１例合并失神发作。合并手足搐搦２例，智能减退４例，小脑症状１例，锥体外系症状１例。血钙１．１３－１．５８ｍｍｏｌ／Ｌ，血磷１．０９－２．０８ｍｍｏｌ／Ｌ。头颅ＣＴ６例均有对称性基底节，小脑齿状核钙化。     

特发性甲状旁腺机能减退症的神经精神症状及误诊分析

特发性甲状旁腺机能减退症（ＩＨＰ）病因未明,可能与自体免疫反应有关,临床表现复杂多样,诊断须与神经精神病鉴别。本文对１２例ＩＨＰ临床资料进行分析,显示以癫痫为首发症状者５例、有精神症状者７例、有肢体麻木者７例;误诊为癫痫５例、神经症２例、血管性痴呆２例、精神分裂症１例,总误诊率８３％。作者认为误诊原因有神经精神症状首发、对ＩＨＰ认识不足、症状发现不典型等。     

特发性甲状旁腺功能减退症神经系统症状的临床观察

我院 1993年以来收治特发性甲状旁腺功能减退症 8例 ,现分析如下。1　临床资料1 1　一般资料　 8例中男 5例 ,女 3例。年龄 2 5～ 60岁 ,平均3 8岁。均无佝偻病及骨软化症 ,无慢性肾脏疾病 ,无腹泻及碱中毒引起的血钙降低 ,无颈部手术及放射治疗病史 ,无体态畸形。有一例有家族病史 ,其兄 6岁时患癫痫并发感染死亡。1 2　神经系统症状　 (1)癫痫 7例 ,占 87 5 %。其中以癫痫为首发症状且误诊为原发性癫痫者 2例。其中全身性发作 3例 ,部分性发作 3例 ,颞叶癫痫 1例。 (2 )手足搐搦 7例 ,占 87 5 %。表现双指关节直伸 ,拇指内收 ,形如“助产…     

IgG4相关特发性肥厚性硬脑膜炎:一例报告并文献复习

目的 探讨IgG4相关特发性肥厚性硬脑膜炎的临床表现、影像学及组织病理学特点.方法 回顾分析1例IgG4表达阳性的特发性肥厚性硬脑膜炎患者的临床表现、组织学特征,并复习相关文献.结果 男性患者,55岁.以癫痫发作起病,主要表现为发作性左侧肢体抽搐.MRI增强扫描显示局部硬脑膜增厚,并呈线样或结节样强化.大体标本观察硬脑...     

IgG4相关特发性肥厚性硬脑膜炎：一例报告并文献复习

目的探讨IgG4相关特发性肥厚性硬脑膜炎的临床表现、影像学及组织病理学特点。方法回顾分析1例IgG4表达阳性的特发性肥厚性硬脑膜炎患者的临床表现、组织学特征,并复习相关文献。结果男性患者,55岁。以癫痫发作起病,主要表现为发作性左侧肢体抽搐。MRI增强扫描显示局部硬脑膜增厚,并呈线样或结节样强化。大体标本观察硬脑膜明显增厚,质地坚硬;光学显微镜下可见硬脑膜异常增厚,纤维结缔组织增生及大量炎性细胞浸润,以淋巴细胞和浆细胞为主,伴少量嗜酸性粒细胞。免疫组织化学染色大量淋巴细胞表达IgG,部分表达IgG4。糖皮质激素和免疫抑制药治疗有效。结论特发性肥厚性硬脑膜炎是一类病因不明的硬脑膜纤维性炎性病变,部分患者可能是IgG4相关硬化性疾病谱中的一员。     

特发性肥厚性硬脑膜炎临床、实验室检查及影像学特点分析

目的分析6例特发性肥厚性硬脑膜炎(IHP)患者的临床特点及辅助检查特征。以提高对特发性肥厚性硬脑膜炎的认识。方法回顾性分析并总结2014年1月至2017年11月在河南省人民医院神经内科就诊的6例临床诊断为特发性肥厚性硬脑膜炎(IHP)患者的临床、实验室检查结果及影像学特点。结果 6例患者主要临床表现为头痛、颅神经受累、癫痫、精神行为异常和共济失调。实验室检查血沉(ESR)、C反应蛋白(CRP)及脑脊液细胞和蛋白等炎性指标增高。磁共振影像表现为对称或不对称的硬脑膜T1加权等和(或)低信号,T2加权低信号,增强有强化。所有患者均给予激素冲击治疗,预后良好。结论 IHP以头痛及颅神经受累为主要表现,实验室检查炎性指标的升高及影像学硬脑膜增厚强化均有助于临床医师诊断及识别本病。     

Idiopathic hypertrophic pachymeningitis mimicking lymphoplasmacyte-rich meningioma.

A 28-year-old woman with a 6-year history of optic neuropathy and 8 years of hearing loss had enhancing dural lesions around the brain stem and in both internal auditory canals on MRI. Histopathology from cranial procedures performed in 1990 and 1993 was originally interpreted as inflammatory meningioma, now known as lymphoplasmacyte-rich meningioma (LRM). Because the clinical course was more consistent with a relapsing process, the original surgical specimens were restudied with additional immunocytochemical stains. The review led to a pathologic diagnosis of idiopathic hypertrophic pachymeningitis (IHP). IHP and LRM can be confused on both imaging and histopathologic grounds.     

Chronic focal polymyositis in the adult

Four patients with chronic focal polymyositis are described. Treatment with steroids appeared to halt clinical progression. The clinical features in all four cases were so strikingly similar as to constitute a syndrome which can be mistaken for muscular dystrophy or spinal muscular atrophy unless investigated fully.     

Neuropathologically confirmed Alzheimer's disease: clinical diagnoses in 394 cases

In the absence of pathognomonic clinical features, the clinical diagnosis of Alzheimer's disease (AD) remains one of exclusion of other dementias. We investigated the clinical diagnoses among 394 neuropathologically confirmed AD cases in a dementia brain bank. Most patients were correctly diagnosed as AD (348 or 88%). Among the misdiagnosed patients, AD was mistaken for a primary depressive disorder in 14, multi-infarct dementia in 13, Parkinson's disease in nine, and alcoholic dementia in four. The number of misdiagnosed AD patients did not differ between physician specialties but was greater among AD patients with agitation, depression, paranoia, or delusions. This retrospective study suggests that the diagnostic sensitivity for AD is high among a cross-section of practicing physicians and that an important factor in mistaking AD for another illness is unfamiliarity with the potential psychiatric symptoms of AD.     

Effectiveness of challenging behavior IHP objectives in residential settings: a longitudinal study

Effectiveness of challenging behavior Individualized Habilitation Plan (IHP) objectives in residential settings was examined. We evaluated three indicators of successful intervention: discontinuation of challenging behavior IHP objectives, change in challenging behavior over time, and frequency of one-to-one crisis intervention and found little evidence of effective intervention. Less than a fourth of participants had a challenging behavior objective discontinued within a year. There was no significant change in challenging behavior from one annual assessment to the next, although the decrease in asocial challenging behavior approached significance. Frequency of crisis intervention also did not change significantly over time. These findings suggest that most challenging behavior IHP objectives are ineffective in reducing challenging behavior.     

Handwriting posture in left-handers: sex, familial sinistrality and language laterality correlates

Study 1 examined the incidence of inverted (IHP) and noninverted (NHP) hand postures in normal left-handers. Hand posture was found to be reliably classifiable; males employed the IHP significantly more often than females; and IHP was significantly more frequent in FS + than FS ?Ss. In Study 2, Ss, were given a lateralized tachistoscopic color-naming latency task, and in Study 3, lateralized word recognition tasks. The results of these studies suggest that, because of its association with FS, hand posture is not a wholly unique new variable and that hand posture appears less promising than FS for differentiating cerebral asymmetries in left-handers.     

Inverted handwriting posture in left handers is related to familial sinistrality incidence

McKeever (1979) reported that left handers who wrote with an inverted handwriting posture (IHP) had a higher incidence of left handedness in their families than did left handers who wrote with a non-inverted handwriting posture (NHP). Parlow and Kinsbourne (1981), however, failed to find a relationship between handwriting posture (HWP) and positive familial sinistrality (FS+). McKeever had classified his left handed subjects according to whether they had several, one, or no left handers among their first degree relatives and biologically-related second degree relatives. Parlow and Kinsbourne classified subjects as FS- or FS+ on the basis of first degree relatives and did not inquire about the handedness of second degree relatives. In the present study, handedness for writing of all first and second degree relatives was recorded for 216 NIP and 255 IHP left handers. Results showed a significant association of HWP and FS factors when second degree relatives were considered, but not when second degree relative handedness was ignored. The relationship was strongest within the maternal family line, where the proportion of left handers was more than twice as high for the IHP as for the NHP group. These findings suggest that HWP may well be of some importance for a complete understanding of the cerebral organization of left handers.     

Nerve sheath tumors of the sellar region.

Nerve sheath tumors, including schwannomas and neurofibromas, rarely affect the sellar region. The authors report two such cases that were mistaken for pituitary adenomas on the basis of clinical and imaging features.