Intradural Lumbar Disc Herniations Associated with Epidural Adhesion : Report of Two Cases

Intradural lumbar disc herniation (ILDH) is rare. In this report, authors present 2 cases of ILDHs associated with severe adhesion between the dural sac and posterior longitudinal ligament. In a 40-year-old man, ILDH occurred in association with epidural adhesion due to ossification of the posterior longitudinal ligament (OPLL). In other 31-year-old man, ILDH occurred in presence of epidural adhesion due to previous spine surgery.     

Severe Neurological Complications Associated With Tourette Syndrome

BackgroundTics and Tourette syndrome are common problems evaluated by both the general pediatrician and pediatric neurologist. The common comorbidities of tics are well known, but the severe neurological complications are rare and may not be appreciated.MethodsThis is a retrospective case series and literature review.ResultsWe present here four adolescents with Tourette syndrome who had severe neurological complications secondary to motor tics. We provide the history, neurological examination, and radiological findings in addition to a review of previously reported cases of vascular and cervical cord complications associated with violent motor tics.ConclusionsWe highlight the importance of recognizing the presenting signs of these complications early and the need to vigorously treat violent motor tics to prevent significant neurological complications.     

POEMS综合征八例并文献复习

目的描述POEMS综合征的临床特点,以提高对此病的认识。方法报道8例POEMS综合征患者临床表现、实验室检查及治疗方法并复习有关文献。结果8例患者均有周围神经病变,其中4例患者血有M-蛋白,症状复杂多样。对8例患者进行不同尝试的治疗,结果症状有一定改善。结论POEMS综合征是一少见的多系统损害疾病,发病机制不很清楚,常有多发性神经病、脏器肿大、内分泌病、M-蛋白和皮肤改变症状。早期病例易被误诊,临床应认真分析、检查,以提高临床检出率。     

Autoantibody-Mediated Sensory Polyneuropathy Associated with Indolent B-Cell Non-Hodgkin's Lymphoma: A Report of Two Cases

Background and Purpose

Abnormalities of the peripheral nervous system occur in 5% of patients with lymphoma. Polyneuropathy has not been described in patients with mantle-cell and marginal-zone B-cell lymphomas.

Case Report

Two elderly patients with indolent non-Hodgkin''s lymphoma developed a progressive sensory polyneuropathy that was associated with serum autoantibodies directed against asialosyl/sialosyl gangliosides and myelin-associated glycoprotein/sulfated glucuronyl paragloboside, respectively, which are peripheral-nerve antigens. The oligoclonal pattern of these antibodies hinted at a lymphoma-induced immune dysregulation. The neuropathy stabilized clinically during treatment with intravenous immunoglobulin G. B-cell lymphoma was managed with a "watchful waiting" approach.

Conclusions

The concept of antigen-specific, immune-mediated neuropathy associated with slow-growing lymphoma of mature B-cells may be underrecognized. The principle of treating the illness underlying neuropathy may not be always indicated or necessary if risk-benefit and cost-benefit analyses are taken into account.     

Two Cases of Hypersexuality Probably Associated with Aripiprazole

Sexual dysfunction is a common side effect in patients treated with antipsychotics but significant differences exist across different compounds. We report hypersexuality symptoms in two female patients with schizophrenia who were receiving treatment with aripiprazole. The patients experienced more frequent sexual desire and greater sexual preoccupation after taking aripiprazole. We discuss the potential neuro-chemical mechanisms for this and argue that aripiprazole''s unique pharmacological profile, partial agonism with high affinity at dopamine D2-receptor, may have contributed to the development of these symptoms.     

Epileptic Seizures in the 4p- Syndrome: Report of Two Cases

Abstract: We report ictal phenomena in two patients with the 4p – syndrome captured on simultaneous video-EEG monitor. One patient, diagnosed as having partial epilepsy, had complex partial seizures and hemiconvulsive status epilepticus. This was associated with more severe mental retardation. The second patient was diagnosed as having the West syndrome and exhibited tonic spasms with a cluster formation. We conclude that various types of epileptic seizures may occur in patients with the 4p - syndrome, including grand mal and myoclonic seizures.     

Myoclonic Encephalopathy of Infants: A Report of Two Cases of ''Dancing Eyes'' Syndrome

The authors describe two cases of myoclonic encephalopathy of infants showing the characteristic features of 'dancing eyes', somatic myoclonic ataxia and irritability. The findings in these and in other reported cases are discussed with regard to treatment and etiology.

RÉSUMÉ

l' encéphalopathie myoclonique des nourrissons: rapport de deux cas
Les auteurs décrivent deux cas d'encéphalopathie myoclonique des nourrissons qui présentaient les traits caractéristiques des 'yeux dansants', ataxie somatique myoclonique et irritabilityé. Les constatations dans ces cas et dans les autres cas rapportés dans la litérature sont discutées du point de vue du traitement et de l'étiologie.

ZUSAMMENFASSUNG

Myoklonische Encephalopathie bei Kindern: Bericht über zwei Fälle
Die Autoren beschreiben zwei Fälle von myoklonischer Encephalopathie mit den charakteristischen Zeichen der 'tanzenden Augen', einer somatischen myoklonischen Ataxie und Erregbarkeit. Die Befunde bei diesen und anderen Fällen in der Literatur werden diskutiert im Hinblick auf Behandlung und Atiologie.

RESUMEN

Encefalopatla mioclónica del lactante: relación de dos casos
Los autores describen dos casos de encefalopatía mioclónica del lactante que presentan las characterísticas de los 'ojos danzantes', ataxia somática mioclónica e irritabilidad. Se discuten los hallazgos de estos casos y de otros publicados con miras a su tratamiento y etiología.     

The Concept Nosology of Heller's Syndrome: Review of Articles Report of Two Cases

Abstract: Based on a review of 60 historical cases of dementia infantilis its synonyms (i.e., Heller's disease, Heller's dementla, Heller's syndrome disintegrative psychosis) a report of 2 new cases, the concept nosology of Heller's syndrome, the most appropriate generic name for such conditions, were studied. In the age of onset, sex ratio, symptoms a favorable prognosis in terms of life, a group of etiologically diverse conditions called Heller's syndrome differs from adult demeath appears classifiable in pervasive developmental disorders according to DSM-III or DSM-III-R, even though its validity as a clinical syndrome remains to be studied.     

Sneddon综合征2例分析

目的 研究Sneddon综合征（Sneddon syndrome,SS）的临床表现、诊断及治疗,以提高临床对该综合征 的认识,提高诊疗水平。 方法 收集2014年8月1日-2017年6月1日于首都医科大学附属北京天坛医院神经病学中心住院治疗的 Sneddon 综合征患者2例,回顾性分析患者临床资料,总结其临床表现、实验室检查、神经影像学检 查、认知功能评价、神经病理学特征及治疗。 结果 2例患者均出现皮肤广泛网状青斑合并多次缺血性卒中事件,均未发现特异性的血生化及脑 脊液异常。头颅磁共振成像（magnetic resonance imaging,MRI）均可见颅内多发的异常信号,颅内外血 管影像学评价均未发现明显的大血管狭窄。认知功能评价可见患者认知功能均存在下降。行皮肤病 理组织活检,病理结果均符合SS表现。治疗上均采取了抗血小板聚集治疗,对于认知功能下降,予以 改善认知疗法。 结论 S neddon 综合征的诊断要综合考虑患者的临床表现、实验室检查、影像学检查结果以及皮肤 病理组织活检结果。患者的特征性皮肤表现及多次卒中事件有助于该病的诊断,必要时可行皮肤活 检以明确诊断。     

脊髓灰质炎后综合征的临床表现（附3例报道）

目的:研究脊髓灰质炎后综合征(PPS)的临床表现。方法:评价3例PPS患者的临床表现。结果:PPS的主要表现是新出现的肌无力、疲劳、疼痛和肢体及躯干缓慢进展的运动神经损害。PPS患者既往有脊髓灰质炎的病史,有部分或完全的神经功能恢复,有长达数十年的神经功能稳定期并除外其他疾病。结论:PPS是一种感染脊髓灰质炎后出现的缓慢进展性的运动神经元综合征。     

Neurological Manifestations of Medical Child Abuse

BackgroundMedical child abuse occurs when a child receives unnecessary and harmful, or potentially harmful, medical care at the instigation of a caretaker through exaggeration, falsification, or induction of symptoms of illness in a child. Neurological manifestations are common with this type of maltreatment.ObjectivesWe sought to review common reported neurological manifestations that may alert the clinician to consider medical child abuse. In addition, the possible sequelae of this form of child maltreatment is discussed, as well as practice recommendations for establishing the diagnosis and stopping the abuse once it is identified.MethodsA review of the medical literature was conducted regarding the reported neurological presentations of this entity.ResultsNeurological manifestations of medical child abuse include false reports of apparent life-threatening events and seizures and reports of induction of symptoms from poisoning. Failure to correlate objective findings with subjective complaints may lead to unnecessary and potentially harmful testing or treatment. This form of child maltreatment puts a child at significant risk of long-term morbidity and mortality.ConclusionsA wide variety of neurological manifestations have been reported in cases of medical child abuse. It is important for the practicing neurologist to include medical child abuse on the differential diagnosis.     

Endovascular Treatment of Aneurysms Associated with Fenestrated A1 Segment of Anterior Cerebral Artery: Report of Two Cases

Fenestration in A1 segment of anterior cerebral artery is a rare entity. Treatment of aneurysms derived from a fenestrated artery may be more challenging because the fenestrations provide specific difficulties. A thorough radiologic work‐up driven by high clinical suspicion is needed. Endovascular treatment, although it has been tried only once, ⁷ appears to be the treatment of choice. We successfully treated 2 cases using endovascular techniques and can conclude that endovascular treatment is a safe and effective treatment of aneurysms associated with this rare variation in the A1 segment of ACA.     

Treatment with L-DOPA in Two Cases of Organic Brain Syndrome

(1) In both of the two cases associated with organic brain syndrome, which were presumably based on the frontal lobe damages, outstanding clinical improvements were observed by oral administration of L-DOPA, 200 to 400 mg daily. (2) Case 1 showed marked depressive symptoms, such as emotional weakness, depressive mood, autistic tendency, apathy and lack of spontaneity due to severe frontal lobe damage by an automobile accident. Treatment with L-DOPA improved his psychic symptoms. He acknowledged his thanks for the L-DOPA treatment by his words that he felt he had regained his former self. When the drug was withdrawn, psychotic symptoms reappeared. (3) Case 2, who was associated with depressive psychic symptoms after carbon monoxide poisoning and whose pneumoen-cepholographs indicated the presence of frontal lobe atrophy, showed remarkable elevation in his mood and in his activity level following administration of L-DOPA. Psychic alterations were most outstanding 14 to 15 days after the commencement of L-DOPA administration and the degree of elation tended to settle down corresponding to the decrease in the dosage. It can be concluded that L-DOPA is also potent in some cases of suppressed emotion due to organic brain damages. A decrease in stimulation from damaged frontal lobe projected to noradrenergic system of the hypothalamus may cause hypofunction of the system and subsequently reflect the state of hypotonic affective disturbance. Administration of L-DOPA may accelerate the synthesis of dopamine function to the normal level.     

Melkersson-Rosenthal综合征(附3例报告)

目的 探讨Melkersson-Rosenthal综合征（MRS）的临床特点及发病机制。方法 分析本院近10年来收治的3例完全型MRS患者临床及随访资料。结果 3例患者临床资料符合完全性MRS的诊断，即间歇性面瘫、再生性唇面肿及皱襞舌，其中1例有家族性皱襞舌。结论 MRS发病机制可能与免疫功能异常所致组织炎性肉芽肿有关，激素治疗有效。     

大脑空气栓塞2例报告

正1病例介绍病例1,女,61岁。主因"发现意识不清5 h"经急诊以"急性脑梗死"于2017年3月27日19:50收入院。入院5 h前,患者在飞机上(睡眠中)被家属发现意识不清,呼之不应,伴汗出、口角流涎,有发作性双上肢肢体不自主抖动。120接诊途中患者出现呕吐,呕吐物为胃内容物,无四肢抽搐、口吐白沫等症状。至入院前,患者神志不清,大便未解,小便正常。既往史:"乳腺癌术后"3年,否认高血压