Behavioral Variant of Frontotemporal Dementia and Homicide in a Historical Case.

Criminal behavior is a clinical feature of the behavioral variant of frontotemporal dementia (bvFTD), ranging from socially inappropriate behavior and minor offenses (such as shoplifting, driving-related violations, housebreaking, trespassing) to the more extreme acts of sex crimes and violence. To our knowledge, no homicide case involving bvFTD is well illustrated in the scientific literature, and only a few anecdotal annotations are available about bvFTD and homicide. This is surprising considering the inclination of individuals with bvFTD to lack impulse control, to manifest disinhibition, to display diminished emotional awareness and loss of empathy, and to show behavior indicative of disordered moral reasoning. Here, we describe the 19th-century homicide case of Benjamin Reynaud, a man whose clinical characteristics suggest the bvFTD diagnosis. Reynaud's case may represent a rare instance of homicide committed by an individual with bvFTD and provide a basis for some reflections regarding the relationship between homicidal behavior and bvFTD.

Frontotemporal Dementia.

Frontotemporal dementia (FTD) is a heterogeneous disorder with distinct clinical phenotypes associated with multiple neuropathologic entities. Presently, the term FTD encompasses clinical disorders that include changes in behavior, language, executive control, and often motor symptoms. The core FTD spectrum disorders include behavioral variant FTD, nonfluent/agrammatic variant primary progressive aphasia, and semantic variant PPA. Related FTD disorders include frontotemporal dementia with motor neuron disease, progressive supranuclear palsy syndrome, and corticobasal syndrome. In this article, the authors discuss the clinical presentation, diagnostic criteria, neuropathology, genetics, and treatments of these disorders.

Kraepelin's description of chronic mania: a clinical picture that meets the behavioral variant frontotemporal dementia phenotype.

Chronic mania is an under-investigated condition and few reports have associated this disorder with an organic background. The present work examines Kraepelin's reliable description of chronic mania from a current behavioral neurology viewpoint. Kraepelin had described a cluster of symptoms that are now recognized as core manifestations of the behavioral variant frontotemporal dementia (bvFTD) clinical phenotype. We also carried out additional reviews of original manuscripts from Kraepelin's peers, in order to find any case reports that might fulfill the current diagnostic proposal for bvFTD. Even though we failed to find an ideal case, we found some scholars who seemed to agree that chronic mania should be considered a special form of dementia. The present work highlights, through historical data, the possible overlapping features between primary psychiatric disorders and neuropsychiatric symptoms secondary to neurodegenerative conditions.

Kraepelin's description of chronic mania: a clinical picture that meets the behavioral variant frontotemporal dementia phenotype / A descrição de Kraepelin de mania crônica: um quadro clínico que corresponde ao fenótipo da variante comportamental da demência fronto-temporal

ABSTRACT Chronic mania is an under-investigated condition and few reports have associated this disorder with an organic background. The present work examines Kraepelin’s reliable description of chronic mania from a current behavioral neurology viewpoint. Kraepelin had described a cluster of symptoms that are now recognized as core manifestations of the behavioral variant frontotemporal dementia (bvFTD) clinical phenotype. We also carried out additional reviews of original manuscripts from Kraepelin’s peers, in order to find any case reports that might fulfill the current diagnostic proposal for bvFTD. Even though we failed to find an ideal case, we found some scholars who seemed to agree that chronic mania should be considered a special form of dementia. The present work highlights, through historical data, the possible overlapping features between primary psychiatric disorders and neuropsychiatric symptoms secondary to neurodegenerative conditions.

RESUMO A mania crônica constitui uma condição subinvestigada e alguns trabalhos têm associado esta desordem a um substrato orgânico. O presente manuscrito analisa a descrição fidedigna de Kraepelin de mania crônica a partir de um ponto de vista atual da neurologia comportamental. Concebemos que ele havia descrito um conjunto de sintomas que atualmente é reconhecido como manifestações centrais do fenótipo clínico da variante comportamental da demência frontotemporal (bvFTD). Também realizamos uma revisão adicional de manuscritos originais de pares contemporâneos de Kraepelin, a fim de procurar por um único relato de caso que poderia preencher critério diagnóstico atual de bvFTD. Mesmo que não tenhamos conseguido encontrar um caso perfeitamente exemplar, identificamos que alguns estudiosos da época pareciam concordar que a mania crônica devesse ser considerada uma forma especial de demência. O presente trabalho destaca por meio de dados históricos a sobreposição entre transtornos psiquiátricos primários e sintomas neuropsiquiátricos secundários a doenças neurodegenerativas.

The undiscovered syndrome: Macdonald Critchley's case of semantic dementia.

Semantic dementia is a unique clinicopathological syndrome in the frontotemporal lobar degeneration spectrum. It is characterized by progressive and relatively selective impairment of semantic memory, associated with asymmetric antero-inferior temporal lobe atrophy. Although the syndrome became widely recognized only in the 1980s, descriptions of cases with typical features of semantic dementia have been on record for over a century. Here, we draw attention to a well documented historical case of a patient with features that would have fulfilled current consensus criteria for semantic dementia, as reconstructed from the notes made by her neurologist, Macdonald Critchley, in 1938. This case raises a number of issues concerning the nosology of the semantic dementia syndrome and the potential value of archived case material.

[Hirotaka Tanabe].

Hirotaka Tanabe was a Japanese neuropsychiatrist engaged in neuropsychological research on cerebrovascular disease and dementia. He contributed widely to the symptomatology of dementia, especially in the field of frontotemporal dementia (FTD). He focused on clarifying the clinical features of a language disturbance, termed Gogi-aphasia by Imura (1943), in 7 patients with anterior temporal circumscribed atrophy. He attributed the nature of Gogi-aphasia to a selective impairment of semantic memory for words and proposed that the pathological process of lobar atrophy with temporal predominance might affect the semantic memory system. In addition, he described in detail the behavioral symptoms of FTD. In his later years, he adovocated a neuropsychological approach to psychiatry.

[John R Hodges].

John Russell Hodges is an English behavioral neurologist, who is mainly engaged in neuropsychological research for neurocognitive disorders. He contributed to symptomatology of transient global amnesia (TGA), semantic dementia, and behavioral variant frontotemporal dementia (bvFTD). Extensive features of his research work are based on his excellent clinician's approach and his outstanding neuropsychological viewpoint, always keeping that neural basis of the symptoms using neuroimaging and neuropathological techniques, and concerning cure and care for patients. Collaboration with psychiatrists, neuroradiologists, neuropsychologists, neuropathologists, social workers, occupational therapists, research nurses, molecular biologists have supported his broad research work. Karalyn Patterson who is an outstanding clinical and experimental neuropsychologist has been particularly important guiding light for his clinical research. He is an excellent clinician and also looks like a commander of a large and well organized research group.

[From Arnold Pick's original descriptions to frontotemporal dementia: the present enlightened by the past an historical approach]. / Des observations originales d'Arnold Pick à la démence frontotemporale : regard sur le présent à partir du passé Un aperçu historique.

Since its seminal characterization, Pick's disease (PiD), was marked by a double ambiguity. Sometimes, with reference to original Pick's descriptions, PiD was macroscopically defined by a circumscribed frontotemporal cerebral atrophy, independently of its causes. Sometimes, on the other hand, it was histologically characterized by absence of Alzheimer pathology and considered as a special form of degenerative process. However, this special degenerative process was defined either by the particular topography of neuronal loss or by specific histological changes, which were described by Alzheimer. Therefore, individualization of PiD and its relationships with Alzheimer's disease have long been controversial, particularly in the United States, despite the clarification provided by French authors in the 1960s, Swiss authors in the 1970s and Swedish and English authors in the 1980s. The term of Frontotemporal dementia (FTD) was choosed by the last authors in 1994 to avoid the debates on the definition of PiD, and to characterize a particular degenerative process without Alzheimer pathology or other diseases. However, the definition and nature of FTD currently remain ambiguous because the term FTD is applied either to clinical syndromes with different presentations according to predominant frontal or temporal atrophy, and regardless of histologic pathology, or to a particular degenerative process, hereditary or sporadic. Notwithstanding neuropathologic and genetic advances in the last decades, significant challenges remain for FTD therapeutic research as a result of the degenerative processes complexity and lack of parallelism between clinical, neuropathological, and genetic data.

Frontotemporal dementia: implications for understanding Alzheimer disease.

Frontotemporal dementia (FTD) comprises a group of behavioral, language, and movement disorders. On the basis of the nature of the characteristic protein inclusions, frontotemporal lobar degeneration (FTLD) can be subdivided into the common FTLD-tau and FTLD-TDP as well as the less common FTLD-FUS and FTLD-UPS. Approximately 10% of cases of FTD are inherited in an autosomal-dominant manner. Mutations in seven genes cause FTD, with those in tau (MAPT), chromosome 9 open reading frame 72 (C9ORF72), and progranulin (GRN) being the most common. Mutations in MAPT give rise to FTLD-tau and mutations in C9ORF72 and GRN to FTLD-TDP. The other four genes are transactive response-DNA binding protein-43 (TARDBP), fused in sarcoma (FUS), valosin-containing protein (VCP), and charged multivesicular body protein 2B (CHMP2B). Mutations in TARDBP and VCP give rise to FTLD-TDP, mutations in FUS to FTLD-FUS, and mutations in CHMP2B to FTLD-UPS. The discovery that mutations in MAPT cause neurodegeneration and dementia has important implications for understanding Alzheimer disease.

Episodic memory in frontotemporal dementia: a critical review.

This review offers a critical appraisal of the literature on episodic memory performance in frontotemporal dementia. Historically, description of patients diagnosed with what was then known as Pick's disease included the presence of memory deficits and an underlying amnestic syndrome was noted in some of these patients. Over the last 20 years, however, the clinical view has been that episodic memory processing is relatively intact in the frontotemporal dementia syndrome. In particular, patients with the subtypes of behavioural variant frontotemporal dementia and progressive non-fluent aphasia are reported to perform within normal limits on standard memory tests. In the third clinical presentation of frontotemporal dementia, semantic dementia, relatively intact episodic memory against a significantly impaired semantic memory was regarded as the hallmark. This position was instrumental in the development of clinical diagnostic criteria for frontotemporal dementia in which amnesia was explicitly listed as an exclusion criterion for the disease. The relative intactness of episodic memory, therefore, appeared to be a useful diagnostic marker to distinguish early frontotemporal dementia from Alzheimer's disease, in which early episodic memory disturbance remains the most common clinical feature. We argue that recent evidence questions the validity of preserved episodic memory in frontotemporal dementia, particularly in behavioural variant frontotemporal dementia. In semantic dementia, a complex picture emerges with preservation of some components of episodic memory, notably recognition-based visual memory and recall of recent autobiographical events. We propose a critical synthesis of recent neuropsychological evidence on retrograde and anterograde memory in light of neuroimaging and neuropathological findings, demonstrating involvement of medial temporal structures in frontotemporal dementia, structures known to be critical for episodic memory processing. We further argue that the multifactorial nature of most memory tests commonly used clinically fail to capture the memory deficits in frontotemporal dementia and that sensitive assessment tools of memory are needed. Together, recent clinical and experimental findings and the historical evidence represent a strong case for a re-evaluation of the importance of memory disturbance in the clinical diagnosis of frontotemporal dementia.

The madness of Dionysus -- six hypotheses on the illness of Nietzsche.

BACKGROUND: Friedrich Nietzsche (1844-1900) is considered as one of the most influential modern thinkers of the last two centuries. The great philosopher and poet developed a mental illness at the age of 44 and died at the age of 56. Pathological examination was not undertaken. At that time Nietzsche was diagnosed as having atypical paralysis progressiva, however recently five other probable diagnoses appeared in literature. METHOD: Literature search in MEDLINE and Web of Science on the illness of Nietzsche. RESULTS: Six hypotheses were identified: 1. Paralysis progressiva (General paralysis of the insane) 2. Bipolar affective disorder followed by vascular dementia 3. Hereditary form of frontotemporal dementia 4. Brain tumor 5. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) 6. Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome. CONCLUSION: Developments in neurology and molecular genetics give new perspectives to the secret of Nietzsche's illness and also there is a consensus on the questioning of the original paralysis progressiva concept. As there was no postmortem, the clinical speculations on the medical problems of the great philosopher remain a challenge.

The birth and early evolution of the frontotemporal dementia (FTD) concept.

An historical overview of the development of the concept of frontotemporal dementia is presented, regarding the last 30 years, using as a backbone the conferences held on this theme, with a start in 1986 in Lund, Sweden. Since then, a dramatic increase in research activities and publications has rapidly expanded our knowledge in this field, a step necessary for the ultimate goal to find an effective treatment of this devastating disorder.