[Non-aphasic misnaming: a case report and literature review].

A 71-year-old, right-handed woman was admitted to our hospital due to a sudden difficulty with conversation. On admission, she was alert, but had a euphoric mood, disorientation, and a disturbance of recent memory. Her speech was fluent. Her repetition and auditory word cognition were excellent, but she had a slight difficulty with naming visual objects. She frequently showed word-finding difficulty and irrelevant paraphasia during free conversation and a word fluency task. Her irrelevant paraphasia was observed more frequently when she was asked to explain her outbreak of anger at the hospital, i.e., it was situation-dependent. She also had anosognosia. MRI showed an infarct in the territory of the left tuberothalamic artery. Single-photon emission computed tomography revealed low-uptake lesions in the left thalamus and orbital frontal, medial frontal, and medial temporal lobes. The patient was diagnosed with non-aphasic misnaming. The clinical characteristics of patients with non-aphasic misnaming in the literature were reviewed. All of the patients with non-aphasic misnaming had word-finding difficulty and irrelevant paraphasia. Additionally, they had either emotional disturbance or anosognosia.

Anosognosia en la enfermedad de Alzheimer: prevalencia, factores asociados e influencia en la evolución de la enfermedad / Anosognosia in Alzheimer disease: Prevalence, associated factors, and influence on disease progression

Introducción: La anosognosia es frecuente en la enfermedad de Alzheimer (EA). El objetivo fue describir su prevalencia en el momento del diagnóstico y analizar los factores predisponentes y su influencia en la evolución posterior de la EA. Métodos: Estudio observacional, multicéntrico, prospectivo, analítico, realizado en consultas de neurología general. Se incluyó a pacientes recién diagnosticados de EA (criterios NINCDS-ADRDA). Se realizaron 2 evaluaciones -cognitivas, funcionales y neuropsiquiátricas-, con un intervalo de 18 meses. Se empleó la Clinical Insight Rating scale como medida de anosognosia (CIR, rango 0-8). El criterio de progresión fue un incremento en la Clinical Dementia Rating-sum of boxes mayor a 2,5 puntos. Las variables predictoras se analizaron mediante regresión logística. Resultados: Se incluyó a 127 pacientes, 94 completaron las 2 evaluaciones. El 31,5% mostraba anosognosia grave (CIR 7-8), el 39,4% conciencia alterada (CIR 3-6) y el 29,1% conciencia normal (CIR 0-2). La mediana del CIR basal en la cohorte fue 4 (Q1-Q3: 1-7) y a los 18 meses 6 (Q1-Q3: 3-8); p < 0,001. La edad avanzada (odds ratio [OR] 2,43; IC del 95%, 1,14-5,19), menor escolaridad (OR 2,15; IC del 95%, 1,01-4,58) y mayor afectación neuropsiquiátrica (OR 2,66; IC del 95%, 1,23-5,74) fueron variables predictoras de anosognosia. El CIR basal fue similar en los grupos con y sin progresión clínica significativa. Conclusiones: La gran mayoría de los pacientes con EA en el momento del diagnóstico muestran un grado significativo de anosognosia que se asocia a mayor edad, menor escolaridad y mayor afectación conductual. No se demostró influencia de la anosognosia sobre la evolución inicial de la EA tras el diagnóstico

Introduction: Anosognosia is a frequent symptom in Alzheimer disease (AD). The objective of this article is to describe prevalence of this condition at time of diagnosis and analyse any predisposing factors and their influence on disease progression. Methods: Observational, prospective, and analytical multi-centre study in an outpatient setting. Patients recently diagnosed with AD (NINCDS-ADRDA criteria) were included. Each patient underwent two cognitive, functional, and neuropsychiatric assessments separated by an interval of 18 months. The Clinical Insight Rating Scale was employed as a measure of anosognosia (CIR, scored 0-8). Progression was defined as an increase in the Clinical Dementia Rating Scale-sum of boxes of more than 2.5 points. The predictor variables were analysed using binary logistic regression. Results: The study included 127 patients, and 94 completed both assessments. Of the total, 31.5% displayed severe anosognosia (CIR 7-8); 39.4%, altered level of consciousness (CIR 3-6); and 29.1%, normal awareness (CIR 0-2). The median baseline CIR in this cohort was 4 (Q1-Q3: 1-7), and at 18 months, 6 (Q1-Q3: 3-8), P<.001. Advanced age (odds ratio (OR) 2.43; CI 95%:1.14-5.19), lower educational level (OR 2.15; CI 95%:1.01-4.58), and more marked neuropsychiatric symptoms (OR 2.66; CI 95%:1.23-5.74) were predictor variables of anosognosia. Baseline CIR was similar in the groups with and without significant clinical progression. Conclusions: The large majority of patients with AD at the time of diagnosis showed significant anosognosia, and this condition was associated with advanced age, lower educational level, and more marked behavioural symptoms. Our results did not show that anosognosia had an effect on the initial clinical progression of AD after diagnosis

Auditory agnosia as a clinical symptom of childhood adrenoleukodystrophy.

OBJECTIVE: To investigate detailed auditory features in patients with auditory impairment as the first clinical symptoms of childhood adrenoleukodystrophy (CSALD). SUBJECTS AND METHODS: Three patients who had hearing difficulty as the first clinical signs and/or symptoms of ALD. Precise examination of the clinical characteristics of hearing and auditory function was performed, including assessments of pure tone audiometry, verbal sound discrimination, otoacoustic emission (OAE), and auditory brainstem response (ABR), as well as an environmental sound discrimination test, a sound lateralization test, and a dichotic listening test (DLT). The auditory pathway was evaluated by MRI in each patient. RESULTS: Poor response to calling was detected in all patients. Two patients were not aware of their hearing difficulty, and had been diagnosed with normal hearing by otolaryngologists at first. Pure-tone audiometry disclosed normal hearing in all patients. All patients showed a normal wave V ABR threshold. Three patients showed obvious difficulty in discriminating verbal sounds, environmental sounds, and sound lateralization and strong left-ear suppression in a dichotic listening test. However, once they discriminated verbal sounds, they correctly understood the meaning. Two patients showed elongation of the I-V and III-V interwave intervals in ABR, but one showed no abnormality. MRIs of these three patients revealed signal changes in auditory radiation including in other subcortical areas. CONCLUSION: The hearing features of these subjects were diagnosed as auditory agnosia and not aphasia. It should be emphasized that when patients are suspected to have hearing impairment but have no abnormalities in pure tone audiometry and/or ABR, this should not be diagnosed immediately as psychogenic response or pathomimesis, but auditory agnosia must also be considered.

Anosognosia and neglect respond differently to the same treatments.

Different techniques, such as optokinetic stimulation, adaptation to prismatic shift of the visual field to the right, or transcutaneous electrical nerve stimulation (TENS), have been shown to alleviate neglect, at least temporarily. We assessed the effect of these techniques on anosognosia and whether their therapeutic effect, if any, matches that on neglect. The effect of the three types of treatment on anosognosia and neglect was investigated in five patients presenting with both severe anosognosia and neglect. Patient 1 was treatment responsive to anosognosia but not to neglect, whereas patients 4 and 5 showed the reverse pattern, i.e., they were treatment responsive to neglect but not to anosognosia. This "treatment response bias" proved to be a valid means to investigate different effects of treatments in the same patient.

Impaired perception of harmonic complexity in congenital amusia: a case study.

This study investigates whether congenital amusia (an inability to perceive music from birth) also impairs the perception of musical qualities that do not rely on fine-grained pitch discrimination. We established that G.G. (64-year-old male, age-typical hearing) met the criteria of congenital amusia and demonstrated music-specific deficits (e.g., language processing, intonation, prosody, fine-grained pitch processing, pitch discrimination, identification of discrepant tones and direction of pitch for tones in a series, pitch discrimination within scale segments, predictability of tone sequences, recognition versus knowing memory for melodies, and short-term memory for melodies). Next, we conducted tests of tonal fusion, harmonic complexity, and affect perception: recognizing timbre, assessing consonance and dissonance, and recognizing musical affect from harmony. G.G. displayed relatively unimpaired perception and production of environmental sounds, prosody, and emotion conveyed by speech compared with impaired fine-grained pitch perception, tonal sequence discrimination, and melody recognition. Importantly, G.G. could not perform tests of tonal fusion that do not rely on pitch discrimination: He could not distinguish concurrent notes, timbre, consonance/dissonance, simultaneous notes, and musical affect. Results indicate at least three distinct problems-one with pitch discrimination, one with harmonic simultaneity, and one with musical affect-and each has distinct consequences for music perception.

A comparative case study of face recognition: the contribution of configural and part-based recognition systems, and their interaction.

Understanding the interaction between the configural and part-based systems in face recognition is the major aim of this study. Specifically, we established whether configural representation of faces contribute to aspects of face recognition that depend on part-based processes, such as identifying inverted or fractured faces. Using face recognition tasks that require part-based or configural processing, we compared the results of CK--a man who has object agnosia and alexia [Moscovitch, M., Winocur, G., & Behrmann, M. (1997). What is special about face recognition? Nineteen experiments on a person with visual object agnosia and dyslexia but normal face recognition. Journal of Cognitive Neuroscience, 9(5), 555-604] but normal upright face recognition, to those of DC--a man who has prosopagnosia but normal object recognition. CK was normal at recognizing faces if configural processing was sufficient, but poor at recognizing faces that were modified so as to alter their gestalt, and require part-based processing (Moscovitch et al.). DC was impaired at recognizing upright faces and his performance declined in all tasks involving recognition of modified faces, including those that depend on part-based and on configural processing. Nevertheless, DC was normal on tasks involving perception of generic faces and face imagery. These results show that although configural face perception can proceed without part-based processing, the reverse is not the case. Our results suggest that the configural system is always necessary for face recognition, and appears to support what remains of face identification even in prosopagnosic people who have an intact part-based system.