Suspected neurodevelopmental disorders in adult patients of memory clinics: Start at the beginning. GREDEV proposals for clinical practice.

Neurodevelopmental disorders are frequent but underestimated in adult populations, even though the cognitive profile of those affected remains atypical throughout adulthood and the disorders can cause significant impairment in activities of daily living. Retrospective diagnosis in this population is challenging. In this article, the GREDEV (working group for the assessment of neurodevelopmental disorders in adults) proposes a brief screening questionnaire for patients with suspected neurodevelopmental disorders, a checklist to facilitate taking the patient history, a list of self-administered questionnaires, and the different key steps of diagnosing neurodevelopmental disorders in adults.

Exploration of verbal and non-verbal semantic knowledge and autobiographical memories starting from popular songs in Alzheimer's disease.

BACKGROUND: In mild Alzheimer's disease (AD), a deficit in episodic memory, particularly autobiographical memory, is clearly established. Several recent studies have also shown impaired semantic memory from the onset of the disease. Musical memory capacities may be especially preserved and listening to music might encourage autobiographical recall. The aim of this study was to explore recall of popular songs in AD. METHODS: We tested 12 patients with mild AD and 12 control subjects. We created a tool made up of old French popular songs: POP 10. This tool is a questionnaire composed of several subtests: melodic free recall, chorus free recall, melodic recognition, chorus recognition, semantic knowledge, autobiographical recall about the song, and autobiographical recall about the interpreter. RESULTS: We used non-parametric tests, the Mann-Whitney test (M-W), the Friedman test, and the a posteriori Wilcoxon test. Results of AD patients were rather similar to those of control participants for melodic memory. Concerning chorus memory (except recognition), semantic knowledge, and autobiographical recall about the interpreter, results of AD patients were significantly weaker than those of control participants. The most important result concerned autobiographical recall about the song: we found no impairment-related differences between the two groups. CONCLUSION: Our findings demonstrate that popular songs can be excellent stimuli for reminiscence, such as the ability to produce an autobiographical memory related to a song. Thus, we confirm that musical semantic knowledge associated with a song may be relatively preserved in the early stages of AD. This leads to new possibilities for cognitive stimulation.

[Diagnosis of psychocognitive complaints or disorders after de novo consultation in a university hospital specialized memory unit]. / Situations de plaintes ou de déficits psychocognitifs observées lors d'une première consultation de mémoire en CM2R.

Psychiatric diagnoses are frequent in memory units, but most neurologists do not feel comfortable about making the diagnosis of psychopathologic cognitive complaint or disorder. The full diagnosis usually requires careful history taking and a neuropsychological examination followed by a clear joint explanation to the patient. There are no good validated clinical signs to distinguish organic memory complaints from psychological disorders, but a nonorganic pattern, as seen in somatic conversion disorder, can be suggestive of a "cognitivoform" disorder. Cognitive doubt is a frequent symptom of anxiety. Bradypsychia is a frequent symptom of depression. We report 50 patients attending their first neurological memory consultation in university hospital for whom a de novo diagnosis of psychopathologic disorder was established on the basis of the clinical setting, observation, examination and neuropsychological tests. These psychopathologic disorders accounted for 40.3% of first-consultation diagnoses. In 76% of cases, the neuropsychological examination was normal. Nine subjects had mild cognitive impairment, concerning executive functions in six and several domains in three. Simulation with atypical neuropsychological pattern was distinguished in three patients. The diagnoses were:psychosis (n=9), traumatic stress (n=5), depression (n=7), anxiety or obsession/compulsion (n=13), hypochondria and "cognitivoform" disorders (n=13). In this study, few patients consulted with a known psychiatric diagnosis. Psychiatric co-morbidity was common. In the memory unit, listening carefully to ascertain the psychic, somatic and social situation of each individual patient appears to be as important as evaluating cognition. The neurologist can rule out organic disorders or an exaggeration of somatic symptoms and determine the precise etiology in order to build a rationale for treatment. The neurologist can also avert an overconsumption of complementary explorations. In conclusion, this study shows first that psychopathologic disorders are commonly encountered in a neurological memory unit, emphasizing the need for training for the neurologist and collaboration with a psychiatrist, and secondly that the role of the memory unit cannot be limited to the diagnosis of Alzheimer's disease.

[Pure progressive amnesia, isolated for 16 years with focal hippocampus atrophy]. / Amnésie pure progressive isolée pendant 16ans et atrophie focalisée de l'hippocampe.

INTRODUCTION: Pure progressive amnesia is a rare and unusual syndrome involving long preservation of autonomy and absence of progression in other cognitive domains. CASE REPORT: We report a case which remained quiescent for 16 years characterized by severe isolated episodic amnesia and preservation of spatial, semantic and implicit memory and autonomy. MRI revealed bilateral focal atrophy of the hippocampus. CONCLUSION: This specific pattern of impairment differs from other types of amnesic syndromes. It is important to identify this kind of amnesia because of its specific course. Studying the topography of the brain lesions may contribute to a better understanding of the neural systems involved in declarative memory.

[Diagnosis contribution of biomarkers in Alzheimer disease: A case of frontotemporal dementia]. / Apport diagnostic des marqueurs biologiques de la maladie d'Alzheimer (MA) dans une observation de dégénérescence lobaire fronto-temporale (DLFT).

INTRODUCTION: Cerebrospinal fluid biomarkers are a fundamental contribution for the diagnosis of Alzheimer disease, in particular for young people. CASE REPORT: We report a case of Alzheimer disease of a 51-years old man whose clinical presentation corresponded to frontotemporal behavioural criteria. The clinical evaluation noticed some troubles in verbal memory. Only the CSF biomarkers confirmed the diagnosis. CONCLUSION: There are different phenotypes of presenile Alzheimer disease and among them we note frontotemporal dementia. It is very important to identify them to introduce a specific treatment.

[N-methyl-d-aspartate receptor antibody encephalitis: a treatable disorder involving B-lymphocytes. A report of two patients]. / Encéphalite à anticorps anti-récepteur du NMDA : une entité traitable impliquant les lymphocytes B. Deux observations.

INTRODUCTION: N-methyl-D-aspartate receptor antibody (anti-NMDA-r AB) encephalitis has been recently identified. We report two cases illustrating the clinical features, response to immunomodulatory treatment and involvement of B-lymphocytes that characterizes this disorder. CASE REPORTS: These patients illustrated the classic clinical features of anti-NMDA-r AB encephalitis including occurrence in young female, presence of severe neurological and psychiatric manifestations with confusion, seizures, mutism, hypovigilence and involuntary movements, and inflammatory cerebrospinal fluid. Both patients improved after immunotherapy. In case 1, the encephalitis was associated with an ovarian teratoma containing neuronal elements. In case 2, there was no tumor identified. A brain biopsy showed prominent perivascular B-cells infiltrates with some T-cells distributed in the brain parenchyma. CONCLUSION: Anti-NMDA-r AB encephalitis is certainly not rare and needs to be promptly recognized and treated. An associated neoplasia is inconstant and the pathophysiology involves humoral immunity.