From Progressive Nonfluent Aphasia to Corticobasal Syndrome: A Case Report of Corticobasal Degeneration

In a previous report, we presented longitudinal clinical, cognitive and anatomical data of a right-handed woman, whose clinical picture evolved from progressive nonfluent aphasia with apraxia of speech to corticobasal syndrome (CBS) in the last stage of the disease. The patient died at age 57 and pathological examination revealed severe atrophy in the left frontal operculum and left premotor area. On histological examination, there was diffuse tau-positive pathology in gray and white cortical hemispheric gray and white matter, basal ganglia and substantia nigra, compatible with corticobasal degeneration (CBD). This case demonstrates the clinical overlap between frontotemporal lobar degeneration and CBD. In this case, early motor speech impairment predicted earlier and more accurately than CBS the presence of underlying tau-pathology and CBD.     

A Case Report of Reiter's Syndrome with Progressive Myelopathy

Reiter''s syndrome belongs to the family of spondyloarthropathies that usually present with a triad of arthritis, urethritis, and uveitis. The diagnostic criteria include clinical, radiological, and genetic findings, and the response to treatment. Nervous system involvement in Reiter''s syndrome is extremely rare. We report here on a 36-year-old man who initially presented with progressive cervical myelopathy and was diagnosed as Reiter''s syndrome 2 years later. The myelopathy was stable after treatment with methotrexate and sulfasalazine. This case suggests that Reiter''s syndrome can present as progressive myelopathy and should be considered in the differential diagnosis of treatable myelopathies.     

进行性智力下降、失语、四肢活动不灵一例

1病例简介患者,女,46岁,因“进行性智力下降、失语、四肢活动不灵3年,加重半年”于2005-05-28入院。患者3年前无明显诱因出现全身水肿,晨起眼睑水肿明显,曾有一过性记忆力丧失,约30min自行恢复。当时因双下肢水肿、行走困难至外院就诊,彩色超声示:双下肢深静脉血栓形成。患者住院1个月后双下肢水肿消失,行走情况好转出院。出院后患者     

A Case Study of Primary Progressive Aphasia: Improvement on Verbs After rTMS Treatment

This case-report shows that high frequency repetitive Transcranial Magnetic Stimulation (hf-rTMS), applied to the left prefrontal cortex, may improve the linguistic skills in Primary Progressive Aphasia (PPA). The patient's performance was evaluated on a battery of language production and memory span tasks, before and after two hf-rTMS treatments and one SHAM treatment. We observed a significant and lasting improvement of the patient's performance on verb production following the application of hf-rTMS versus Baseline and SHAM conditions. This finding suggests that hf-rTMS may directly strengthen the neural connections within an area of metabolic dysfunction and encourages the use of rTMS as an alternative therapeutic tool for neurodegenerative forms of aphasia.     

成人型Leigh综合征临床、影像学与神经病理学研究(附一例家系报告)

目的总结成人型Leigh综合征临床、影像学与神经病理学特点,并探讨该病发病机制和诊断标准。方法分析1例Leigh综合征的临床表现、MRI特征和脑组织神经病理特点。结果患者II-4男,32岁,出现共济失调15年,智能障碍10年,脑干症状3个月,32岁死亡。其兄Ⅱ-2,13岁发病,表现为共济失调、痉挛步态伴智能损害,27岁死亡。Ⅱ-4患者MRI-T2加权像可见双侧壳核、尾状核、丘脑、中脑及桥脑被盖对称性高信号病灶;行尸检神经病理检查示,前述部位有多发对称性变性坏死,伴髓鞘脱失、胶质增生和毛细血管增生。结论Leigh综合征可于成人期发病,除临床病程特征外,成人型Leigh综合征与婴幼儿型Leigh综合征在临床、影像学与神经病理学特点诸方面具有相似性,成人患者出现Leigh样症候群应考虑成人型Leigh综合征的可能。     

Nothing to Say,Something to Sing: Primary Progressive Dynamic Aphasia

We describe a 76-year-old man (ADY) with dynamic aphasia in the setting of a degenerative frontal lobe dementia: primary progressive dynamic aphasia. He displayed a striking paucity of propositional speech despite intact speech production, and preserved singing and prosody. Vocal expression in the verbal and musical domains was investigated in a series of neuropsychological experiments based on novel language and musical tasks that were designed to establish the nature and specificity of the verbal output deficit. The features of the language disorder indicated that the speech output pathway was disrupted at the early stage of generation of a new pre-verbal message. In contrast, tests of musical output demonstrated that the generation of new musical ideas was unimpaired. The domain-specificity of dynamic aphasia may result from the disruption of specific cognitive processes necessary for the creation of verbal messages, as well as selective damage of brain regions involved in language production.     

Klinefelter's Syndrome and Epileptic Psychosis: A Case Report

Abstract: We present here a case of Klinefelter's syndrome with epileptic psychosis. The patient was a 30-year-old male, who suffered from epilepsy for 23 years. He showed auditory hallucination, delusion of reference and delusion of grandeur. At present there are some cases of Klinefelter's syndrome with various psychiatric symptoms, but there are few cases of Klinefelter's syndrome with epileptic psychosis. These various neuropsychiatric symptoms of the present case are thought to be associated with a specific effect of chromosomal abnormality through the cerebral dysfunction.     

可逆性后部脑病综合征一例

1病例简介患者,男性,58岁。因“头痛、视物不清、抽搐伴意识障碍1 d”于2005年3月30日入院。患者1 d前无明显诱因出现后枕部剧烈疼痛,伴视物不清,后突然意识丧失,伴双眼上视、口吐白沫、四肢抽搐,持续约2 min,抽搐自行缓解,发作时伴尿失禁。此后意识障碍     

肢带综合征1例及文献复习

目的:探讨淋巴瘤肌肉侵犯的临床特点。方法:报道1例肌肉病理确诊为淋巴瘤合并肌肉侵犯患者,对其临床、肌肉MRI表现和肌肉活检病理结果结合复习文献予以分析,总结淋巴瘤肌肉侵犯的临床特点。结果:淋巴瘤肌肉侵犯发生率较低,主要表现为受累肌肉肿块、疼痛和无力。MRI中可表现为局部肿块或弥漫性肿大及异常信号。肌肉活检及免疫组化有助于与肌炎鉴别和淋巴瘤的分型。以肌肉侵犯为主要表现的淋巴瘤化疗以CHOP方案为主,预后较好。结论:淋巴瘤肌肉侵犯可引起包括肢带综合征在内的肌病表现,有肌肉肿块或无力症状以及相应MRI表现的病例应考虑淋巴瘤肌肉侵犯可能。     

Kleine-Levin Syndrome: A Case Report and Review of Literature

BackgroundKleine-Levin syndrome presents with recurrent hypersomnia along with a number of other neuropsychiatric features, of which hyperorality has not been described frequently.MethodWe report a male adolescent who presented with recurrent hypersomnia, hypersexuality, and hyperorality. Magnetic resonance imaging of the brain and overnight polysomnography followed by a multiple sleep latency tests were ordered. Excessive daytime sleepiness was assessed with the Epworth Sleepiness Scale.ResultsMagnetic resonance imaging of the brain did not reveal any abnormality. Overnight video-synchronized polysomnography and multiple sleep latency tests ruled out narcolepsy. Epworth Sleepiness Scale score at baseline was 22. He was prescribed lithium carbonate 300 mg twice a day. The symptoms improved within a week after starting lithium carbonate therapy.ConclusionKleine-Levin syndrome may present with hyperorality, and our patient responded well to lithium carbonate therapy.     

Acquired Epileptiform Opercular Syndrome: A Second Case Report, Review of the Literature, and Comparison to the Landau-Kleffner Syndrome

Summary: A 5-year-old girl developed recurrent prolonged episodes of severe oral apraxia, dysarthria, and drooling, similar to the opercular syndrome in children. Each episode lasted several weeks to >6 months and was associated with exacerbation of epileptiform activity in her EEG. Electrographic status epilepticus during slow wave sleep (ESES) was recorded during three of the exacerbations. The EEG improved markedly when clinical symptoms subsided. Antiepileptic drugs (AEDs) were not effective, although there was some improvement when they were combined with a ketogenic diet. A similar case was described by Roulet et al. We believe that this is a distinct epileptic syndrome, equivalent to the Landau-Kleffner syndrome (LKS).     

Neurology：眶梗死综合征1例

正2016年,David Dongkyung Kim在Neurosurgery上发表了一篇眶梗死综合征(orbital infarction syndrome)的病例报告。患者,男性,76岁。既往有头痛、头皮痛、咀嚼障碍3个月。本次表现为右眼上睑下垂,完全眼肌麻痹,瞳孔散大固定,角膜水肿,右侧三叉神经V1分布区感觉减退。颞动脉活检证实为巨细胞动脉炎。计     

阿帕替尼导致可逆性后部脑病综合征1例

正1病例介绍患者女性,56岁,主因"头痛伴间断性抽搐6 d"以症状性癫痫于2016年8月26日收入血管神经病学病区。患者于6 d前无明显诱因出现头痛、视物模糊,继而出现意识丧失、四肢抽搐、双眼上翻、口角向右歪斜,持续5 min后症状缓解,予镇静对症治疗,期间抽搐发作6次。后患者无抽搐发作,但头痛持续不缓解,遂收入院     

Ideomotor and Ideational Apraxia in Corticobasal Degeneration: A Case Study

Corticobasal degeneration (CBD) is a progressive disorder that can be characterised by asymmetrical akinetic rigidity, involuntary movements, cortical sensory loss, alien limb syndrome and asymmetrical apraxia (Gibb et al., 1989; Rinnie et al., 1994). Diagnosis of praxic disabilities is thought to be essential for distinguishing CBD, in its early stage, from other akinetic-rigid syndromes. However, the nature of apraxia in CBD, and the relations between ideomotor and ideational apraxia, are not well understood. For example, if there is an ideational deficit in a given patient, does this deficit occur independently of any ideomotor disorder, or are the two impairments linked in some manner? In the present paper we report a case study of a patient with apraxia due to CBD. We examine whether the disorder is confined to production tasks, or whether there is also a related deficit in recognising the correct actions performed with objects (an ideational deficit). We also evaluate whether a disorder found for action with single objects dissociates from the ability to link multiple actions into more complex, everyday tasks. The performance of our patient showed an impairment in both action production and action recognition system, suggesting a component of ideational as well as ideomotor apraxia in CBD.     

A Case of Cerebral Erdheim-Chester Disease With Progressive Cerebellar Syndrome

Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis. Cerebellar involvement is rare in this syndrome. We report a 37-year-old woman with slowly progressive cerebellar ataxia, dysmetria of limbs, nystagmus, and dysarthria, bilateral painful axillary masses, and generalized arthralgia. Brain MRI revealed cerebellar atrophy with focal lesions in the pons, middle cerebellar peduncle, and the cerebellum. She underwent incisional biopsy of her axillary masses which showed findings consistent with ECD. An MRI of her lower extremities revealed lesions in the diaphyses, metaphyses, and epiphyses of the proximal tibia and distal femur bilaterally. This is a rare case of cerebral ECD with progressive cerebellar syndrome associated with cerebellar atrophy.     

Clozapine Withdrawal Catatonia and Neuroleptic Malignant Syndrome: A Case Report

Catatonia as a clozapine withdrawal syndrome has not been documented. We report a case of excited catatonia with fever, autonomic instability, and delirium—a picture of malignant catatonia (lethal catatonia) after abrupt clozapine withdrawal. The use of conventional neuroleptics transformed the excited malignant catatonia into a stuporous state resembling neuroleptic malignant syndrome (NMS). Such a transformation of excited lethal catatonia into NMS has been described in the literature, providing support for the hypothesis that NMS is a variant of catatonia. Opinions, however, have been conflicting whether lethal catatonia and NMS are indistinguishable. We argue that NMS may be regarded as a neuroleptic-induced retarded (stuporous) subtype of malignant catatonia, clinically indistinguishable from nonneuroleptic retarded malignant catatonia but different from the excited form. To differentiate between the two subtypes of malignant catatonia would help resolve the controversy. The nosological status of excited catatonia, a poorly studied condition, remains unclear. The two subtypes of catatonia may differ in pathophysiology and responses to treatment. Clinicians should be alert to catatonia as a possible clozapine withdrawal phenomenon, and excited catatonia deserves more research attention.