The left central gyral lesion and pure anarthria]

We report a very rare case of pure anarthria with lesion analysis. A 44-year-old right-handed man suffered from a cerebral infarction with a mild right hemiparesis and speech disturbance. An MRI of the brain 1.5 months post onset revealed a lesion confined to the left central gyrus. One month after the onset, his spontaneous speech was dysprosodic and laborious. It was contaminated with dysarthria and phonological paraphasias. However, language comprehension, repetition and naming abilities were normal. Most remarkably he showed no impairment in writing with his left hand. Over the following months, his difficulties in verbal output showed general amelioration, but the isolated impairment in the domain of articulation characterized by dysprosody, dysarthria, and phonological paraphasia persisted. As for the symptomatology of pure anarthria resulting from precentral gyral lesions, there have been controversies about its pureness. Some argue that the so called pure anarthria always shows some degree of writing disturbances, albeit mild in degree. Others maintain there certainly exists the pure type without any signs of agraphia. In the present case lesions were limited to the central gyrus but spared the lowest opercular portion. The previous reports of pure anarthria that had mild agraphia all had lesions involving the opercular portion. We conclude the sparing of this area is most likely related with sparing of writing capacity in pure anarthria.     

Pure Anarthria with Predominantly Sequencing Errors in Phoneme Articulation: A Case Report

A 77-year-old left-handed man presented with pure anarthria following cerebral infarction. The lesion was restricted to the right precentral gyrus extending to the immediately underlying subcortical white matter and the frontal part of the insular cortex. Qualitative analysis of anarthria revealed that half of the phonemic-articulatory errors in spontaneous speech were sequencing ones. Sequential errors were detected at the phoneme level in both consonants and vowels, and at the syllable level. Most of the sequential errors were pre-positioning. Sequential errors were observed both within and across words. In clear contrast with anarthria, writing and comprehension was preserved, which suggested the problem was limited to oral expression. Our findings provide further support that the precentral gyrus and/or the insular cortex of the language dominant hemisphere is responsible for the temporal sequencing of the articulatory programming.     

Frontotemporal dementia with ubiquitinated neuronal inclusions and visuospatial impairment

Dementia with ubiquitinated neuronal inclusions has been described only with frontotemporal dementia (FTD). The authors report a patient with progressive FTD accompanied by prominent impairments in visuospatial cognitive functions. Pathology was characterized by ubiquitin-positive intranuclear and cytoplasmic neuronal inclusions. Cortical pathology was widespread and posteriorly accentuated but spared the hippocampal dentate gyrus.     

Pure Word Deafness with Possible Transfer of Language Dominance

Abstract: A 55-year-old, right-handed male patient wtih a past history of a stroke followed by a difficulty of speech and hearing fell and manifested a left hemiplegia. He could neither comprehend spoken language and melody nor repeat them, though he spoke with paraphasia and understood written language and nonverbal sound. An electroencephalogram, pneumoencephalogram and cerebral angiogram suggested the existence of old infarcts in the left temporal lobe and a probable new one in the right cerebrum. A diagnosis of this case was made as pure word deafness which might be caused by a reimpairment of the language function possibly transferred to the nondominant, right hemisphere following the early stroke.     

Frontotemporal dementia: Its definition,differential diagnosis,and management

Background: Frontotemporal dementia (FTD) is defined as primary neurodegeneration of the anterior temporal and/or frontal lobes resulting in a group of associated conditions marked by changes in cognition, language, personality, and social functioning. FTD was previously thought to be a rare disease. However, researchers report that FTD is the third most common form of dementia. Because adults with FTD have deficits in language, cognition, and behaviour, familiarity with FTD subtypes, associated deficits, and currently available management strategies is warranted.

Aims: The aims of this tutorial are (a) to define frontotemporal dementia including behavioural and language characteristics of the three clinically distinct FTD subtypes (frontotemporal variant, nonfluent progressive aphasia, semantic dementia); (b) to identify similarities and differences between FTD and Alzheimer's dementia; and (c) to discuss management strategies for patients with FTD.

Main Contribution: Different subtypes and presentations of FTD as well as the neurological, behavioural, and language symptoms that have been consistently identified are reviewed. Behavioural and language symptoms of the two FTD subtypes with primary language disturbances (nonfluent progressive aphasia and semantic dementia) are also reviewed. Patients with FTD are frequently misdiagnosed as presenting with Alzheimer's dementia due to limitations in the literature describing the differing profiles of the two populations. When considering neurological changes, behavioural changes, language and communication behaviours, and disease progression, these patient populations are distinct and easily differentiated. Finally, management strategies are discussed. Although there is no cure for FTD, medical intervention can address some of the associated symptoms, and behavioural techniques may manage the client's environment and prolong communication abilities.

Conclusions: General discussion seeks to differentially diagnose FTD dementia from Alzheimer's dementia as well as to clarify the language and communication symptoms of FTD subtypes. Future research directions are suggested for developing evidence‐based direct and indirect management strategies.     

Pure word deafness with possible transfer of language dominance

A 55-year-old, right-handed male patient with a past history of a stroke followed by a difficulty of speech and hearing fell and manifested a left hemiplegia. He could neither comprehend spoken language and melody nor repeat them, though he spoke with paraphasia and understood written language and nonverbal sound. An electroencephalogram, pneumoencephalogram and cerebral angiogram suggested the existence of old infarcts in the left temporal lobe and a probable new one in the right cerebrum. A diagnosis of this case was made as pure word deafness which might be caused by a reimpairment of the language function possibly transferred to the nondominant, right hemisphere following the early stroke.     

Word deafness as a cortical auditory processing deficit: a case report with MEG

Pure word deafness is a rare disorder dramatically impairing comprehension of spoken language, while auditory functions remain relatively intact. We present a 71-year-old woman with a slowly progressive disturbance of speech perception due to pure word deafness. MRI revealed degeneration of the temporal lobes. A magnetoencephalographic investigation using alternating single tone stimulation showed that N100 was followed by a second transient response and was abnormally prolonged up to 600-700 ms. We conclude that auditory processing is disturbed at long latency ranges following the N100, which may result in the clinical presentation of pure word deafness.     

The neuropathology of FTD associated With ALS

There is increasing recognition of a clinical overlap between frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Recent advances in our understanding of the neuropathologic, biochemical, and genetic basis of these conditions provides evidence for a common underlying pathogenesis. The neuropathology in most cases of FTD with ALS is a subtype of frontotemporal lobar degeneration, characterized by neuronal inclusions that are immunoreactive for ubiquitin but not tau (frontotemporal lobar degeneration with ubiquitinated inclusions). These cases show significant pathologic overlap with clinically pure FTD and those with classic ALS. Moreover, the ubiquitinated pathologic protein in all these conditions has recently been identified as TDP-43. A number of families have been reported with autosomal dominant FTD-ALS linked to chromosome 9p and these also have TDP-43-positive frontotemporal lobar degeneration with ubiquitinated inclusions pathology. Together, these findings suggest that FTD-ALS is part of a clinicopathologic spectrum of disease, now identified as TDP-43 proteinopathies.     

Crossed anarthria and a dissociated lateralisation of language

INTRODUCTION: Crossed anarthria cases are uncommon and rather old. OBSERVATION: We report the case of a right-handed 55-year-old man who presented crossed pure anarthria due to a hemorrhage in the premotor cortex (feet of F1 and F2) and in the high part of Pierre-Marie's quadrangle. CONCLUSION: The study of different tasks (articulation, verbal fluency, direct object word-generation from a verb) showed a dissociated lateralisation of his language. Lexico-semantic and grammatical tasks are processed in the left hemisphere. Articulation programming occurs in the right hemisphere.     

Focal brain lesions and language dysfunction

To be a neurologist, training in cognitive/behavioral neuroscience is essential. In this review article, the author tries to emphasize the localization relationship between focal brain lesions and language dysfunction, by discussing from split-brain syndromes, hemispheric asymmetry, to pure word dumbness, pure word deafness (verbal auditory agnosia), pure agraphia, and pure word blindness (pure alexia; visual word agnosia), and finally to aphasia and aprosodia in general. Hopefully, after getting familiar with all the terms, the readers will feel free exploring language disturbances and behavioral neurology for their own interests.     

Frontotemporal dementia can be distinguished from Alzheimer's disease and subcortical white matter dementia by an anterior-to-posterior rCBF-SPET ratio

Sixteen patients with frontotemporal dementia (FTD), 27 with early-onset Alzheimer's disease, 25 with late-onset Alzheimer's disease, 19 with subcortical white matter dementia (SWD) and 28 normal controls underwent semiquantitative regional cerebral blood flow measurement (rCBF) using single-photon emission tomography (SPET; (99m)Tc-HMPAO) and either computerized tomography (CT) or magnetic resonance imaging (MRI) of the brain. An anterior-to-posterior rCBF-SPET ratio (mesial superior frontal gyrus/medial temporal lobes) was calculated, which significantly separated the FTD group from the other dementia groups and controls with a sensitivity of 87.5% and a specificity of at least 78.6%. CT/MRI was found to be helpful in the differential diagnosis between FTD and SWD. In FTD patients, the mesial superior frontal gyrus, near the polus frontalis, was found to be the region with the most reduced rCBF values.     

Neuropsychological patterns in right versus left frontotemporal dementia.

Patients with frontotemporal dementia (FTD) often present with an asymmetric left or right-sided anterior cerebral perfusion abnormality that is associated with differential behavioral symptoms. However, whether patients with primarily right versus left FTD also have unique neuropsychological characteristics has not been previously investigated. Comparisons of 11 patients with right-sided FTD and 11 with left FTD indicated that the 2 patient groups showed relatively distinct cognitive profiles. Patients with right FTD exhibited relatively worse performance on PIQ than VIQ, and on select nonverbal executive tasks relative to their verbal analogs (e.g., design fluency < word generation; Picture Arrangement < word sequencing). In contrast, patients with left FTD showed the opposite pattern. In addition, the 2 patient groups differed on several absolute test scores; patients with right FTD demonstrated more errors and perseverative responses, and worse percent conceptual level responses, on the Wisconsin Card Sorting Test, while the left FTD patients obtained significantly worse scores on the Boston Naming Test, and Stroop word reading and color naming. Verbal and nonverbal memory, mental speed, visual perceptual-constructional skill, and IQ subtest scaled scores did not significantly differ between groups. These data indicate that FTD should not be viewed as a unitary disorder, and that neuropsychological testing holds promise for the differential diagnosis of right versus left FTD.     

Cortical thickness in frontotemporal dementia, mild cognitive impairment, and Alzheimer's disease

Cortical thickness analysis has been proposed as a potential diagnostic measure in memory disorders. In this retrospective study, we compared the cortical thickness values of 24 patients with frontotemporal dementia (FTD) to those of 25 healthy controls, 45 symptomatic subjects with stable mild cognitive impairment (S-MCI), 15 subjects with progressive mild cognitive impairment (P-MCI), and 36 patients with Alzheimer's disease (AD). The patterns of regions of thinning in FTD when compared to controls and also S-MCI patients showed similar trends; thinning of the bilateral frontal poles and bilateral medial temporal lobe structures, especially the anterior part of the gingulum, the uncus, and parahippocampal gyri. Cortical thinning in FTD was also found on the boundary regions of parietal and occipital lobes. In the P-MCI group compared to FTD, the trend of thinning in small distinct areas of the parietal and occipital lobes was observed. The FTD and AD groups did not differ statistically, but we found trends toward thinning in FTD of the left cingulate gyrus, and the left occipitotemporal gyri, and in AD of the inferior parietal, occipitoparietal, and the pericalcarine regions, more in the right hemisphere. In FTD, increased slowness in the executive test (Trail-Making A) correlated with the thinner cortex, whereas the language tests showed the lower scores, the thinner cortex in the left hemisphere. Cortical thickness might be a tool for detecting subtle changes in brain atrophy in screening of dementia prior to the development of diffuse or lobar atrophies.     

Art and the brain: the influence of frontotemporal dementia on an accomplished artist

A talented artist developed a progressive aphasia syndrome associated with frontotemporal dementia (FTD). As her disease progressed, language and executive skills declined, but her paintings became freer and more original. She demonstrates that artistic development can occur in the setting of language-dominant types of FTD. The study of artistic development in the setting of FTD suggests that language is not required for, and may even inhibit, certain types of visual creativity.     

Initial symptoms, survival and causes of death in 115 patients with frontotemporal lobar degeneration

The early and differential diagnosis of the clinical phenotypes of frontotemporal lobar degeneration (FTLD), including frontotemporal dementia (FTD), semantic dementia (SD) and non-fluent progressive aphasia (NFPA), can be challenging. It may be difficult not only to differentiate these conditions from normal aging, psychiatric disorders, and other dementias, but also to distinguish between them. For early diagnosis, information on the initial and presenting symptoms of the FTLD phenotypes is essential. In the present study caregivers of 78 patients with FTD, 20 patients with SD and 17 patients with PA were interviewed about initial symptoms. In patients with FTD, the most frequent initial symptoms were alterations of personality, followed by forgetfulness and word finding difficulty. Patients with SD presented with word finding difficulty and behavioral disturbances. Almost all patients with PA developed word finding difficulty as the first manifestation of their disorder. Diagnostic latency - the time from disease onset to diagnosis was 4.1 years in FTD, 4.2 years in SD and 3.1 years in PA. Caregivers, and in some cases also patients, should be educated about the likely course and mortality of FTLD. To obtain information about survival time and cause of death associated with FTLD we analyzed follow-up data on 106 patients of whom 25 had died. The median survival time from the occurrence of first symptoms was 14 years. Mortality risk was significantly higher in patients with an early disease onset. Causes of death were varied, but pneumonia and sudden unexplained deaths were particularly frequent.     

Language disorders in Landau-Kleffner syndrome

In the present long-term study, we analyzed language disorders in four patients with Landau-Kleffner syndrome. Their common first symptoms were disability in understanding spoken words, followed by inarticulation and a decreased amount of speech. All patients showed auditory verbal agnosia to some degree at some stage of their illness. However, one patient showed typical sensory aphasia as the first symptom, and another patient showed nonverbal auditory agnosia followed by pure word deafness. Thus, patients with Landau-Kleffner syndrome show sequential and sometimes hierarchical language disorders beginning with sensory aphasia, followed by auditory agnosia, and finally word deafness during their disease process. During long-term follow-up (20 to 30 years), all patients showed marked recovery in language without any intellectual handicap, but with some disability in spoken language, auditory verbal perception, and a discrepancy between Wechsler Verbal and Performance IQ scores.     

Cortical ubiquitin-positive inclusions in frontotemporal dementia without motor neuron disease: a quantitative immunocytochemical study

Ubiquitin-positive tau-negative inclusions were initially described in the rare form of frontotemporal dementia (FTD) associated with motor neuron disease. However, recent studies have indicated that these inclusions are also present in typical FTD, which is usually characterized by nonspecific histological changes. To examine the contribution of these inclusions to neuronal loss and to explore their relationship with disease duration, we performed a quantitative immunocytochemical analysis of 38 typical FTD cases. Relationships between neuron and ubiquitin inclusion densities as well as between duration of illness and neuropathological parameters was studied using linear regression in both univariate and multivariate models. Ubiquitin-positive tau-negative intracytoplasmic inclusions were present in 65.8% of cases in the dentate gyrus, 57.9% in temporal cortex and 31.6% in frontal cortex. The highest densities of ubiquitin-positive inclusions were consistently observed in the dentate gyrus, followed by the temporal and frontal cortex. There was no statistically significant relationship between neuron and ubiquitin-positive inclusion densities in any of the areas studied. In contrast, ubiquitin-positive inclusion densities in the dentate gyrus were negatively related to the duration of illness. Our data suggest that the development of ubiquitin-related pathology is the rule and not the exception in typical FTD, yet is not causally related to neuronal loss. They also reveal that the development of ubiquitin-positive inclusion densities in the dentate gyrus may be associated with a more aggressive form of the disease.     

Acquired sociopathy and frontotemporal dementia

BACKGROUND: It is not understood why some patients with frontotemporal dementia (FTD) manifest sociopathic behavior. OBJECTIVE: To examine the prevalence and characteristics of sociopathic behavior in FTD patients as compared to those with Alzheimer's disease (AD). METHODS AND PARTICIPANTS: We surveyed a group of FTD patients and a group of AD patients for sociopathic behavior, evaluated the characteristics surrounding their acts, and compared the groups on neuropsychological tests and functional neuroimaging. Twenty-eight outpatients with FTD (15 men, 13 women; 61.9+/-7.1 years; Mini-Mental State Examination score 23.6+/-8.1) were compared with 28 patients with clinically probable AD (13 men, 15 women; 66.1+/-9.2 years; Mini-Mental State Examination score 21.3 +/- 5.3). Main outcome measures included: (a) the prevalence of sociopathic acts; (b) a structured interview; (c) neuropsychological tests including the Frontal Assessment Battery, and (d) clinically obtained positron emission tomography or single photon emission tomography scans. RESULTS: Sixteen (57%) of the FTD patients had sociopathic behavior compared to two (7%) of the AD patients (chi(2)=13.84, p<0.001). Sociopathic acts among FTD patients included unsolicited sexual acts, traffic violations, physical assaults, and other unacceptable behaviors. On interview, the FTD patients with sociopathic acts were aware of their behavior and knew that it was wrong but could not prevent themselves from acting impulsively. They claimed subsequent remorse, but they did not act on it or show concern for the consequences. Among FTD patients with sociopathy, neuropsychological assessment showed impaired motor inhibition, and functional neuroimaging showed right frontotemporal involvement. CONCLUSION: The results suggest that sociopathy in FTD results from a combination of diminished emotional concern for the consequences of their acts and disinhibition consequent to right frontotemporal dysfunction. In many jurisdictions, FTD patients with sociopathy would not pass legal criteria for 'not guilty by reason of insanity'.     

Postmorbid learning of saxophone playing in a patient with frontotemporal dementia

Some patients with frontotemporal dementia (FTD) show an artistic enhancement of musical abilities. However, no patients with FTD, to date, have been reported to be able to learn how to play a musical instrument after disease onset. Herein we describe a patient (J. K.) who had never played any musical instruments premorbidly, but who learned to play the saxophone after being diagnosed with a behavioral variant of FTD. He mastered a repertoire that consisted of 10 pieces of Korean folk songs over a period of three years. Furthermore, his saxophone skills were high enough to outperform other students in his class.