Naming people ignoring semantics in a patient with left frontal damage.

Studies about proper name anomia generally assume that persons' names are harder to recall than other semantic information one knows about them and that name retrieval is not possible without biographical knowledge. We describe a patient, SB, who, after a left frontal haemorrhage, was unable to recall any biographical information about people she could name. Moreover, she had a normal score in an Object Picture Naming Test, but gave confabulatory answers in a Semantic Questionnaire involving the same items. The role of frontal function in producing this pattern of impairment is discussed, together with the possible existence of a direct route from visual perception to proper name retrieval.

Fronto-temporal dementia and motor neuron disease: a neuropsychological study.

The neuropsychological follow-up study of a 58-year-old man suffering from Motor Neuron Disease (ALS/MND) and Fronto-Temporal Dementia (FTD) is reported. Neuromuscular signs first appeared at the age of 51 and slowly progressed to late bulbar involvement; behavioural symptoms of the frontal type first appeared around age 53; lastly, several neuropsychological symptoms suggestive of worsening temporal involvement supervened at age 57. Our patient died at 59 of respiratory failure with the classic clinical and neuroradiological picture of FTD. A short discussion addresses the controversial issue of the coupling of ALS/MND with Dementia and its possible interpretation as the expression of a chance association of relatively common diseases, versus that of a single multifaceted disease. The role of a detailed neuropsychological assessment is highlighted, within the context of increasingly specific diagnostic criteria for FTD.

Degenerative dementia of the frontal type. Clinical evidence from 9 cases.

The retrospective neurological, neuroradiological and neuropsychological observation of 9 cases of dementia with frontal symptoms is reported. Aim of this paper is to contribute to the clinical corpus of data related to the frontal features of the frontotemporal dementia (FTD) syndrome, so as to support clinicians' awareness and widen the information available for the diagnostic approach to the dementias. FTD is a clinical diagnosis which does not imply a single underlying pathology, since more than one condition can induce the syndrome. Inertia and behavioural symptoms were the traits characterizing both the onset and the progression of the disease in our patients. Progression brought to the surface behavioural and neuropsychological patterns generically traced back to frontal dysfunction. Social inadequacy was the most salient trait. From a clinical point of view frontal dementias may be regarded as a conceptually different type of dementia with respect to Alzheimer's disease, i.e., a 'behavioural' as opposed to a 'cognitive' progressive disorder.

Seeing objects smaller than they are: micropsia following right temporo-parietal infarction.

We report the case of an 84-year-old lady who, after a right temporo-parietal infarction, complained of seeing things smaller than she expected. She also related that straight lines appeared distorted and described seeing colours as if they were a badly mixed assemblage of hues. Her visual field was normal except for a transient left field extinction. No spatial neglect emerged. The patient's micropsia remained unchanged during the course of the six month follow-up.

Variables linked to psychotic symptoms in Alzheimer's disease.

This study is aimed at assessing the prevalence and features of psychotic symptoms in a sample of 180 patients with Alzheimer's disease (AD). Sixty-four patients (35.5% of the sample) showed one or more psychotic symptoms. The presence of these symptoms correlated with the severity of the patients' cognitive impairment, as well as the patients' age at first assessement. Sex, duration of illness, education and familiar history for dementia were not significantly correlated with the psychotic symptoms. Hallucination was the most common symptom (24.4%, of the whole sample), followed by delusion (18.3%) and misperception (11.1%). Visual hallucinations were more frequent than auditory, and, among delusions, persecutory and theft themes were largely prevailing. Misperceptions included television related phenomena and phantom boarder illusions. A follow-up study of 37 patients demonstrated the association between the presence of psychotic symptoms and a steeper slope of cognitive deterioration. Copyright 1998 Lippincott Williams & Wilkins

Is memory loss without anatomical damage tantamount to a psychogenic deficit? The case of pure retrograde amnesia.

Following a car accident, a patient remained unconscious for approximately 20 min and confused for a few hours. When he could be questioned, he was found to have lost all past memories. The retrograde amnesia covered his whole life and concerned autobiographic events as well as famous facts and encyclopaedic knowledge. It also partially involved the verbal and visual lexicon. Reading, writing and counting were no longer possible. The profound impairment of retrograde memory contrasted with the preservation of anterograde memory, which permitted the patient to reacquire some of the notions he had lost, without, however, recovering the feeling of a personal experience of autobiographical information. Four years later, the retrograde deficit was unmodified, except for what had been relearnt. The search for data in support of an organic or psychological aetiology was negative. No signs of brain damage were apparent at the neurological examination and on CT, MRI and SPECT. On the other hand, there was no evidence of a psychiatric history, psychological stress or emotional precipitants that could substantiate the hypothesis that the patient derived a primary or secondary gain from amnesia. We propose that cases of focal retrograde amnesia, similar to the present one, deserve to be classified separately from organic and psychogenic forms under the label of 'functional' retrograde amnesia, a syndrome in which the threshold of activation of premorbid memories is abnormally raised by the trauma, leaving the encoding and retrieval of new memories unaffected.

Persistent retrograde, amnesia following a minor trauma.

A 19-year-old man showed dense retrograde amnesia (RA) for autobiographical and public events covering his entire life, following a motor car accident that caused no apparent brain damage. His learning abilities remained excellent and permitted him to recover knowledge of the past, based on information gathered from other people and the media, although he never reacquired the sense of personal experience. At the onset he also showed occasional gaps in his semantic memory, e.g., he failed to recognize a few objects and was unable to provide factual information about sports he had long been playing. Eventually, his amnesia was predominantly restricted to autobiographical events or facts. After 29 months the deficit has remained unchanged. CT, MRI and PET were negative. Psychogenic amnesia was considered, but eventually ruled out for want of any evidence pointing to emotional problems, stressful situations or secondary gains. We posit that for reasons unknown a mild trauma can cause functional inhibition of the access to the engrams that are already stored, leaving intact the ability to encode and retrieve new memories.

Cognitive modelling of face processing: evidence from Alzheimer patients.

This is a prospective neuropsychological study on face processing in Alzheimer's disease (AD). The aim was to assess the prevalence and the nature of face processing disorders in AD, and at investigating possible inter-test dissociations within the framework of currently used face processing models. A standardized four-test battery of unknown face discrimination and familiar face recognition was given to 30 mildly deteriorated patients with AD. Half of the patients performed below the cut-off in at least one of the tests. Deficits in familiar face recognition tests were more frequently observed than deficits in unknown face discrimination tests. There was no correlation between impairment of face processing and overall cognitive impairment or visual disorders. A multiple single case approach allowed us to elicit statistically warranted double dissociations between tasks assessing unknown face discrimination and tasks assessing familiar face recognition. Moreover, the ability to decide whether or not a stimulus is a face or a non-face has proven to be a non-mandatory step to further process the face stimuli. All together, these findings support the hypothesis that distinct pathways are involved in the processing of unknown and familiar faces, as posited by Bruce and Young [Br. J. Psychol. 77, 305-327, 1986].

The 'Petites Madeleines' phenomenon in two amnesic patients. sudden recovery of forgotten memories.

We report two cases of recovery from retrograde amnesia, which occurred almost suddenly, 1 year and 1 month from onset, respectively. Amnesia followed a left thalamic infarction in one patient and a mild head trauma in the other. Full and permanent recovery occurred within a short time after the spontaneous emergence to consciousness of a single autobiographical event, triggered by the specific experience of a very similar new event. Recovery was limited to retrograde amnesia, leaving unaffected the anterograde deficit present in one of the patients. The nature (psychogenic versus organic) of our patients' memory loss is commented upon in the light of a review of previously reported cases of retrograde amnesia. The transient defect of retrograde memory is discussed in terms of a reversible distortion of the neuronal 'patterned matrices' suggested by Gloor (Brain 1990; 113: 1673-94).