桥本脑病二例

目的探讨桥本脑病的临床特点及其治疗方法。方法分析我院收治的2例桥本脑病患者的临床资料。结果两例患者分别以认知功能障碍及癫痫为首发症状,脑脊液检测、脑电图、头颅核磁共振检查未见特异性改变,但是血液甲状腺过氧化物酶抗体(TPOAb)明显增高,例1患者甲状腺功能正常,例2患者有甲亢。2例患者均对皮质类固醇激素敏感。结论临床上有以认知功能障碍、癫痫等症状为突出表现者,须考虑桥本脑病的可能。     

以精神症状起病的桥本脑病1例报告并文献复习

桥本脑病（又称自身免疫性甲状腺炎相关的糖皮质激素敏感性脑病）是与桥本甲状腺炎相关的以神经系统症状为主要表现的疾病,呈急性或亚急性起病,出现癫痫发作、震颤、肌阵挛、共济失调、精神病等表现,有复发及缓解交替过程。以精神症状起病的桥本脑病的诊断和治疗存在分歧,现报道一例桥本脑病患者,建议在临床工作中如果遇到无法解释的脑病时,如果抗甲状腺抗体滴度高,即使甲状腺功能正常也要考虑桥本脑病的可能性。     

桥本脑病2例及文献复习

目的探讨桥本脑病的临床特点,以提高对本病的认识。方法回顾性分析2例确诊桥本脑病患者的临床资料并复习相关文献。结果 2例均为急性起病,1例表现为精神症状及意识障碍,另1例表现为运动及感觉障碍。2例抗甲状腺抗体均增高,抗甲状腺过氧化物酶抗体增高明显,2例脑电图均广泛异常,1例脑脊液蛋白明显升高。2例糖皮质激素治疗均有效。结论桥本脑病临床表现错综复杂,容易漏诊和误诊,当临床上遇到无法解释的中枢神经系统疾病,应常规检查抗甲状腺抗体,确诊后应尽早用糖皮质激素治疗。     

桥本脑病2例及文献复习

目的探讨桥本脑病的临床特点,以提高对本病的认识。方法回顾性分析2例确诊桥本脑病患者的临床资料并复习相关文献。结果 2例均为急性起病,1例表现为精神症状及意识障碍,另1例表现为运动及感觉障碍。2例抗甲状腺抗体均增高,抗甲状腺过氧化物酶抗体增高明显,2例脑电图均广泛异常,1例脑脊液蛋白明显升高。2例糖皮质激素治疗均有效。结论桥本脑病临床表现错综复杂,容易漏诊和误诊,当临床上遇到无法解释的中枢神经系统疾病,应常规检查抗甲状腺抗体,确诊后应尽早用糖皮质激素治疗。     

桥本脑病的临床、影像学及病理学特点(附1例报告)

目的 研究桥本脑病的临床、影像学和病理学特点.方法 回顾性分析1例桥本脑病患者的临床资料.结果 本例患者的主要临床表现为快速进展的认知功能障碍及癫(癎)发作.血清抗甲状腺抗体显著增高,甲状腺功能基本正常.颅脑MRI显示双侧额、顶、颞、枕叶皮质及皮质下多发性长T1、长T2病灶,以皮质更加明显.增强扫描全部病灶均无强化.病理学检查显示反应性胶质细胞、小血管增生及少量淋巴细胞浸润.电镜检查显示神经细胞部分空泡样变性和线粒体肿胀,白质内可见小囊腔形成.经用糖皮质激素和免疫抑制剂治疗,患者的症状和影像学改变均得到了明显的缓解.结论 桥本脑病的临床特点为亚急性大脑皮质功能障碍,血抗甲状腺抗体增高而甲状腺功能正常,糖皮质激素治疗有效.影像学表现为广泛脑皮质及皮质下异常病灶;病理学改变无特异性.     

桥本脑病4例临床特点分析

目的探讨桥本脑病的发病机制、临床表现、实验室检查特点及预后。方法分析本院收治的4例桥本脑病患者的临床资料,观察其临床表现、实验室及影像学检查特点及治疗,并随访1～2年。结果4例患者均为女性,临床表现主要为认知功能障碍、行为异常、抽搐等。实验室检查患者血清抗甲状腺抗体水平明显增高。3例经大剂量糖皮质激素冲击治疗,患者症状较前缓解,随访无复发。结论桥本脑病是一种伴有抗甲状腺抗体水平增高、对激素反应良好的脑病综合征,一般预后良好。     

儿童桥本脑病:病例报道及文献综述

桥本脑病是一种少见的自身免疫系统疾病,在儿童中尤为少见。其临床表现多种多样,诊断主要依靠早期识别临床特征以及甲状腺抗体的检查。本文报道1例以精神行为异常首发症状的儿童桥本脑病病例,结合实验室检查,影像学检查以及治疗进行分析;同时本文总结以往相关文献,对桥本脑病特别是儿童桥本脑病的流行病学、临床表现、实验室检查及影像学特征、诊断及治疗等方面进行讨论。     

桥本脑病临床及实验室分析

目的探讨桥本脑病的临床、实验室、影像学特点。方法回顾性分析2例桥本脑病的临床资料和治疗效果,结合相关文献进行分析。结果 2例患者均有以记忆力下降为主的认知障碍,颅内均有多发病灶。1例有幻视、癫痫发作和明确的血管狭窄,1例有反复头晕、肢体力弱,无血管狭窄。2例均存在抗甲状腺抗体阳性和甲状腺功能亢进,且糖皮质激素治疗有效。结论认知障碍合并抗甲状腺抗体的增高是本病的特点之一,可以合并颅内血管狭窄,确诊后可早期糖皮质激素治疗。     

桥本脑病22例临床分析

目的 探讨桥本脑病临床特点,提高诊断与治疗水平.方法与结果 对22例桥本脑病患者的临床表现、实验室和影像学特点、治疗及预后进行回顾分析,临床主要表现为急性或亚急性发病的认知功能障碍（9例）、精神症状（6例）、癫（痫）发作（5例）、共济失调（5例）、卒中样发作（6例）、锥体外系症状（3例）和意识障碍（3例）,且血清抗甲状腺过氧化物酶抗体（19例）和抗甲状腺球蛋白抗体（20例）水平明显升高.其中21例经糖皮质激素冲击治疗,17例病情不同程度改善.结论 桥本脑病是一种伴抗甲状腺抗体水平升高的脑病,大多数患者对糖皮质激素冲击治疗反应良好,早期诊断、及时治疗对改善患者预后尤为重要.对于不明原因的脑病患者,应考虑桥本脑病的可能,推荐血清抗甲状腺抗体作为常规实验室检查项目.     

桥本脑病22例临床分析

目的探讨桥本脑病临床特点,提高诊断与治疗水平。方法与结果对22例桥本脑病患者的临床表现、实验室和影像学特点、治疗及预后进行回顾分析,临床主要表现为急性或亚急性发病的认知功能障碍(9例)、精神症状(6例)、癫发作(5例)、共济失调(5例)、卒中样发作(6例)、锥体外系症状(3例)和意识障碍(3例),且血清抗甲状腺过氧化物酶抗体(19例)和抗甲状腺球蛋白抗体(20例)水平明显升高。其中21例经糖皮质激素冲击治疗,17例病情不同程度改善。结论桥本脑病是一种伴抗甲状腺抗体水平升高的脑病,大多数患者对糖皮质激素冲击治疗反应良好,早期诊断、及时治疗对改善患者预后尤为重要。对于不明原因的脑病患者,应考虑桥本脑病的可能,推荐血清抗甲状腺抗体作为常规实验室检查项目。     

Autoimmune thyroid encephalopathy presenting with epilepsia partialis continua.

We report the first case of an autoimmune thyroid encephalopathy presenting with multifocal motor status epilepticus. A 37-year-old female patient was admitted with multifocal motor seizures intractable to intravenous status epilepticus treatments, asymmetrical quadriparesis, truncal ataxia and continuous semi-rhythmical jerks. Pathological signal alterations were detected in both precentral cortices in MRI examination. Autoimmune thyroiditis was diagnosed after radiological examinations of the thyroid gland and thyroid function tests. Seizures promptly ceased following intravenous steroid treatment. Immunohistochemistry studies showed mild to moderate neuronal staining with the plasma and CSF samples. Remarkably, autoimmune thyroiditis may present with migrating focal motor status epilepticus. We recommend anti-thyroid antibody screening for multifocal motor status epilepticus cases of unspecified cause.     

Auditory seizures in autoimmune epilepsy: a case with anti‐thyroid antibodies

In its classic presentation, Hashimoto's encephalopathy is an acute‐subacute complex neuropsychiatric syndrome with cognitive impairment, hallucinations, myoclonus, tremor or ataxia, associated with elevated anti‐thyroid antibodies. Corticoids and immunotherapy are dramatically effective. However, in some cases, not all the associated features are presented and this delays diagnosis and appropriate treatment. We describe a man with abrupt onset of recurrent auditory seizures resulting in refractory non‐convulsive status epilepticus. The patient was diagnosed with an autoimmune encephalopathy with elevated serum and CSF anti‐thyroid antibodies. None of the antiepileptic drugs were successful, however, following immune‐modulating therapy, the refractory non‐convulsive status epilepticus dramatically improved, as did the patient overall. We suggest that Hashimoto's encephalopathy should be suspected in otherwise healthy patients with unexplained new‐onset focal recurrent auditory seizures which do not respond to antiepileptic drugs. The presence of anti‐thyroid antibodies in the CSF supports this diagnosis.     

Hashimoto encephalopathy - is it underdiagnosed in pediatric patients?

Hashimoto encephalopathy (HE) is associated with Hashimoto thyroiditis. Clinically it presents with variable symptoms like seizures, neuropsychiatric changes or focal neurological deficits. Autoimmune phenomena are hypothesized for the pathogenesis. HE has mainly been described in the adult population. We present two 14-year-old patients who presented with recurrent seizures and mental decline. SPECT and PET scans showed distinctly pathological changes. Both patients were diagnosed with HE and improved dramatically on steroids. We feel that HE is a rare but important differential diagnosis of encephalopathy also in the pediatric population. As this disease responds well to steroids, we recommend to obtain basic thyroid function tests as well as thyroid antibodies in all cases of unexplained encephalopathy or unexplained status epilepticus.     

Hashimoto's encephalopathy. Case report and literature review

We present a 43-year-old man with recurrent episodes of Hashimoto's encephalopathy who was diagnosed with autoimmune thyroiditis in childhood. Encephalopathy started with subacute dementia followed by extrapyramidal and psychiatric symptoms of insidious onset. He had also status epilepticus which occurred within the first year of the disease. The patient was in euthyreosis, but increased levels of antithyroid antibodies were found. MRI of the brain was normal. Electroencephalography was initially normal and later showed diffuse slowing with generalized theta/delta activity. The cerebrospinal fluid examination revealed a high level of protein which decreased when remission of the disease was achieved. After other etiology was excluded Hashimoto's encephalopathy was diagnosed. Almost complete clinical recovery after steroid administration was observed. Attempts of prednisone withdrawal led to recurrence of neurological and psychiatric symptoms. The diagnosis of Hashimoto's encephalopathy should be considered in each case with subacute encephalopathy associated with high levels of antithyroid antibodies (despite normal thyroid function) and in the absence of other brain diseases.     

Antiglutamic Acid Decarboxylase Antibodies in Children With Encephalitis and Status Epilepticus

Encephalitis may present with seizures or status epilepticus, and the etiology is usually presumed. Specific antibodies to ion channels, receptors, and other synaptic proteins were identified during the past decade. However, only a few studies investigated antiglutamic acid decarboxylase antibodies and antibodies to cell membrane ion channels or surface antigens in pediatric encephalitis and status epilepticus. We examined antibodies to glutamic acid decarboxylase and cell membrane ion channels or surface antigens in acute-phase serum from 17 children with encephalitis and status epilepticus. Antiglutamic acid decarboxylase antibody titers were compared with those of control children manifesting therapy-resistant epilepsy. Antiglutamic acid decarboxylase antibody titers were significantly higher in those with encephalitis and status epilepticus than in those with therapy-resistant epilepsy. No patient demonstrated antibodies to cell membrane ion channels or surface antigens. Six children exhibited positive antiglutamic acid decarboxylase antibodies. One child died, three manifested postencephalitic epilepsy with neurologic deficits, and two recovered well. Higher antiglutamic acid decarboxylase antibody titers were evident in the children with encephalitis and status epilepticus. Clinicians should be aware of the potential role of antiglutamic acid decarboxylase antibodies in children with encephalitis and status epilepticus.     

Complex partial status epilepticus as a manifestation of Hashimoto''s encephalopathy

Epileptic seizures are a frequent manifestation of Hashimoto's encephalopathy. However, status epilepticus associated with Hashimoto's encephalopathy are not well characterized in medical literature. We described here a 16-year-old girl who presented with complex partial status epilepticus associated with elevated anti-thyroid antibodies. Ictal EEG showed lateralized high amplitude rhythmic delta waves over the right hemisphere and ictal single-photon emission computed tomography revealed regional hyperperfusion of the right parietal and temporal lobes. The patient was unresponsive to antiepileptic drug therapy but responded to intravenous steroid treatment. Screening of serum anti-thyroid antibodies for unexplained encephalopathy with epileptic seizures is suggested, as early recognition and prompt steroid treatment may lead to a favorable prognosis.     

Hashimoto encephalopathy causing drug-resistant status epilepticus treated with plasmapheresis

Hashimoto encephalopathy is a rare, clinically heterogenous condition. Its treatment is based on corticosteroids. A previously normal 12-year-old boy was admitted to our pediatric emergency department with status epilepticus. He experienced a recurrence of status epilepticus after pentobarbital withdrawal, and required repeated resumptions of drug-induced coma. He manifested acute personality changes. His limbic encephalitis markers were normal, but his level of anti-thyroid peroxidase antibody was high. A diagnosis of Hashimoto encephalopathy was considered. Our patient responded to plasmapheresis instead of corticosteroid treatment. This case report is the first, to the best of our knowledge, of plasmapheresis because of Hashimoto encephalopathy in a child.     

Encephalopathy associated to autoimmune thyroid disease: a more appropriate term for an underestimated condition?

Hashimoto's encephalopathy is a severe and rather infrequent clinical condition initially described in patients suffering from chronic lymphocytic thyroiditis. Its origin is still controversial but it can be agreed to have an autoimmune etiology. In fact, its most characteristic finding is the high titre of antithyroid antibodies, especially antimicrosomal. We describe three cases of Hashimoto's encephalopathy and establish a relationship between the clinical status, the antithyroid antibody levels and its response to corticosteroid treatment. There was an excellent response to corticosteroid treatment in all three cases. Interestingly, one case was associated with Graves' disease. Given this, and after the review of the literature, we believe that the term 'encephalopathy associated to autoimmune thyroid disease' could be more appropriate to define this entity. Finally, we suggest that autoimmune thyroid encephalopathy must be suspected in the face of unaccounted acute or subacute encephalopathy with high levels of antithyroid antibodies.     

Syndrome of Electrical Status Epilepticus During Sleep: Epileptic Encephalopathy Related to Brain Development

BackgroundEpileptic encephalopathy with electrical status epilepticus during sleep is an age-related and self-limited disorder. The present study analyzed the etiology, demographics, and pathogenesis of patients with electrical status epilepticus during sleep to provide information on the diagnosis and therapy of this syndrome.MethodsThe etiologies of epileptic encephalopathy with electrical status epilepticus during sleep in patients admitted in Chinese People's Liberation Army General Hospital from 2009 to 2014 were retrospectively analyzed. Patients were classified into the genetic, structural-metabolic, and unknown groups according to the etiology. Demographics and clinical characteristics of all the patients were then analyzed and compared among groups.ResultsThe etiologies of epileptic encephalopathy with electrical status epilepticus during sleep in 75 patients mainly included benign childhood epilepsy with centrotemporal spikes, Landau-Kleffner syndrome, polymicrogyria, and migration disorders. Age at onset of epilepsy did not show a specific pattern, but age at onset of epileptic encephalopathy with electrical status epilepticus during sleep was concentrated at age 6-9 years. The mean age at onset of epilepsy in the genetic group was significantly older than that in the structural-metabolic group (P < 0.05). Age at onset of epileptic encephalopathy with electrical status epilepticus during sleep did not significantly differ between the two groups.ConclusionsElectrical status epilepticus during sleep is an epileptic encephalopathy related to brain development and presents an age-dependent occurrence.