汉语失语症测评软件在脑出血后失语评定中的应用

目的探讨中康法汉语标准失语症电脑软件测评系统(CRRCAE)在左侧基底节区脑出血患者语言功能评定及疗效观察中的应用价值。方法对24例左侧基底节区脑出血患者行微创手术治疗,术后行语言康复训练等治疗,并在术前、术后4 w,8 w应用CRRCAE(电脑版)行语言功能评定。结果治疗后与治疗前相比,CRRCAE测评系统各项评分均有明显提高,且随着时间的推移,患者的语言功能明显提高。结论CRRCAE电脑软件测评系统可用于评定脑出血患者语言恢复的程度和特点,能有效的鉴别其改善的程度,具有较好的临床应用价值。     

Breakpoint to MIC quotient: A parameter to rapidly evaluate the in vitro bactericidal activity of β-lactams on Enterobacteriaceae

Minimum inhibitory concentration (MIC) and minimum bactericidal concentration (MBC) do not completely predict the bactericidal nature of the antimicrobial agent. Patients with pathogens having MICs near the clinical breakpoint experience a higher risk of clinical failure. This study defined an indicator, breakpoint to MIC quotient (BMQ), that incorporates MIC and the European Committee on Antimicrobial Susceptibility Testing breakpoint. The BMQ was inversely correlated with MBC in antibiotic combinations against Enterobacteriaceae strains (Spearman coefficient ≤ –0.96). This new parameter may provide timely additional insight for choosing an antibiotic for a severe bacterial infection.     

The genetics of primary progressive aphasia

Background: Primary progressive aphasia (PPA) is a disorder in which language impairment is the initial and predominant symptom. Three main phenotypes are described, the nonfluent variant (nfvPPA), the semantic variant (svPPA) and the logopenic variant (lvPPA). Although PPA is most commonly a sporadic disorder, recent studies have shown an association of PPA with mutations in a number of genes.

Aims: To understand the extent to which PPA may be inherited, which genetic mutations may cause it, and whether the phenotypes of genetic PPA differ from sporadic PPA.

Main Contribution: In around 20–30% of patients with PPA, a family history is present although nfvPPA is more heritable than svPPA and lvPPA which are both usually sporadic disorders. Mutations in the progranulin (GRN) and chromosome 9 open reading frame 72 (C9orf72), genes are the major causes of genetic PPA.

Conclusions: Key pointers that may suggest testing for a GRN mutation in PPA are a family history of one of the disorders within the frontotemporal dementia spectrum, a nfvPPA phenotype, particularly if presenting with a prominent anomia and asymmetrical fronto-temporo-parietal atrophy. In someone with nfvPPA and a family history, GRN should be tested initially but a search for hexanucleotide repeat expansions in the C9orf72 gene should be performed if negative, particularly if there are features of motor neurone disease, or a family history of someone with motor neurone disease. Mutations in other genes are only very rare causes of PPA but if GRN and C9orf72 are both negative, testing for mutations in the microtubule-associated protein tau (MAPT), valosin-containing protein (VCP) and presenilin 1 (PSEN1) should be considered.     

Trouble and repair during conversations of people with primary progressive aphasia

Background: Primary progressive aphasia (PPA) affects a range of language domains that impact on communication. Little is known about the nature of conversation breakdown in PPA. The identification of trouble in conversation, its repair and the success of repairs has been used effectively to examine conversation breakdown in neurogenic language disorders such as dementia of the Alzheimer type (DAT) and acute onset aphasia. This study investigated trouble and repair in the conversations of people with PPA.

Aims: The first aim of this study is to describe the contributions of individuals with PPA and their conversation partner to conversation. The second aim is to describe the trouble that occurs in dyadic conversations between three individuals with PPA and their communication partner. The third aim is to describe the repair behaviours used by the individuals with PPA and their communication partners.

Methods & Procedures: Dyadic conversations about everyday activities between three individuals with PPA and their partners and three control dyads were video recorded and transcribed. Number of words, number of turns and length of turns were measured and trouble-indicating behaviours (TIBs) and repair behaviours were categorised.

Outcomes & Results: Individuals with PPA had reduced mean length of turn but maintained their share of turn-taking. They demonstrated a variety of TIBs that differed from the noninteractive repairs, which do not require a response from the partner in the conversation and which have been observed in studies of conversation in DAT. Their partners bore the greater burden of highlighting trouble and need for repair using collaborative, interactive, TIBs. Three different conversational profiles were observed in the three PPA dyads, reflecting different patterns of language and cognitive impairment.

Conclusions: Individuals with PPA were active participants in conversation effectively indicating and responding to trouble. Understanding trouble and repair in the conversations of individuals with PPA has the potential to enhance assessment and inform clinical practice.     

Motor speech disorders associated with primary progressive aphasia

Background: Primary progressive aphasia (PPA) and conditions that overlap with it can be accompanied by motor speech disorders. Recognition and understanding of motor speech disorders can contribute to a fuller clinical understanding of PPA and its management as well as its localisation and underlying pathology.

Aims: To review the types of motor speech disorders that may occur with PPA, its primary variants, and its overlap syndromes (progressive supranuclear palsy syndrome, corticobasal syndrome, motor neuron disease), as well as with primary progressive apraxia of speech.

Main Contribution: The review should assist clinicians’ and researchers’ understanding of the relationship between motor speech disorders and PPA and its major variants. It also highlights the importance of recognising neurodegenerative apraxia of speech as a condition that can occur with little or no evidence of aphasia.

Conclusion: Motor speech disorders can occur with PPA. Their recognition can contribute to clinical diagnosis and management of PPA and to understanding and predicting the localisation and pathology associated with PPA variants and conditions that can overlap with them.     

The patterns of progression in primary progressive aphasia—Implications for assessment and management

Background: Primary progressive aphasia (PPA) is a progressive language disorder with preserved cognitive function for at least 2 years from onset. The main variants currently distinguished are: non-fluent/agrammatic (nfvPPA), semantic (svPPA), and logopenic (lvPPA). Patients with initial language presentation may subsequently develop other symptoms, such as behavioural dysfunction or apraxia. The clinical pattern of PPA depends on the location of atrophy, the underlying pathology, and the stage of the disease.

Aims: This review aims at characterising longitudinal changes in clinical presentations of different PPA variants and at presenting implications of these changes for the assessment, diagnosis, and management.

Main contribution: The three PPA variants differ not only in terms of language impairment, but also with regard to cognitive and behavioural profile. Apraxia and rigidity frequently occur in the course of nfvPPA. Patients with lvPPA seem to follow the pattern of aphasic Alzheimer’s disease, where language impairment is accompanied by episodic memory deficit. Individuals diagnosed with svPPA often develop behavioural dysfunction similar to that observed in behavioural variant of frontotemporal dementia.

Conclusions: Implications for patient care are dependent on PPA variant and on the stage of the disease. In svPPA, emphasis should be on the management of semantic and behavioural problems in daily life. Caregivers of nfvPPA patients should be informed about the possible emergence of apraxia and other movement disorders. In contrast, families of individuals with lvPPA should be made aware of and trained to cope with an episodic memory decline and possible progression to other varieties of PPA.     

Pediatric Anti-NMDA (N-methyl D-Aspartate) Receptor Encephalitis

BackgroundWe report the clinical features and course of pediatric patients presenting with anti-N-methyl D-aspartate receptor (NMDA-R) encephalitis.MethodsSingle-center 4-year observational study of pediatric encephalitis associated with NMDA-R antibodies in the serum and/or the cerebrospinal fluid.ResultsThree girls with anti-NMDA-R encephalitis were identified. All presented with an acute hyperkinetic movement disorder and seizures, expressive aphasia, and emotional lability requiring inpatient treatment for 1-3 months. Imaging and electroencephalogram findings were nondiagnostic. None had an underlying tumor or ovarian teratoma. All received immune-modulatory therapy, including one or more of the following: high-dose methyl-prednisolone, plasma exchange, intravenous immunoglobulin or mycophenolate mofetil. Two of the three patients relapsed within 6 months of presentation and required retreatment with plasma exchange. All have remained in subsequent remission, with two of the three requiring second-line immunotherapy with rituximab.ConclusionsHyperkinetic movements in pediatric patients presenting with acute encephalopathy and prominent psychiatric symptoms should elicit a search for NMDA-R antibodies early in the evaluation. Relapses require aggressive immunomodulatory treatment for remission. This series highlights a unique positron emission tomography scan finding of hypermetabolism in one of the patients that correlated with her clinical symptoms. Recovery and rehabilitation can be prolonged, often taking years after the initial diagnosis. Early identification and treatment is likely to reduce relapses and limit morbidity associated with this potentially devastating but treatable encephalitis.     

Cerebellar Mutism in Acute Disseminating Encephalomyelitis

BackgroundCerebellar mutism in children occurs after posterior fossa tumor resection and can have lasting effects on cognition, language, and behavior. Cerebellar mutism in acute disseminated encephalomyelitis is rare.PatientA 7-year-old boy with a 3-day history of fever, vomiting, and diarrhea presented with altered mental status and expressive aphasia. Magnetic resonance imaging showed new diffusion restriction in the bilateral dentate nuclei and right cerebellum. With treatment, he began to verbalize again but had long-term cognitive and language difficulties.ConclusionAcute disseminated encephalomyelitis is commonly a benign process, but its effect on the cerebellum can be long-lasting.     

非流畅性失语患者朗读两类成语的研究

目的 考查非流畅性失语患者在语音提示下朗读2种类型成语的能力,为确定其朗读训练方向提供依据.方法 对30例非流畅性失语患者实施自身对照研究,于言语训练前进行成语朗读能力测试.测试时,在语音提示成语前2个字后,分别朗读唯一搭配成语和双重搭配成语后2个字,2种类型成语测试满分分别为30、34分.结果 患者朗读唯一搭配成语成绩（21.12±11.65）分,双重搭配成语成绩（20.84±9.89）分,差异有统计学意义（P＜0.05）.结论 非流畅性失语患者朗读2种类型成语的能力存在差异,测试成绩可作为制定和调整言语训练的参考标准及训练内容.