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Background: Posterior cortical atrophy (PCA) is a progressive dementia characterized by prominent disorders of higher visual processing, affecting both dorsal and ventral streams to cause Balint's syndrome, alexia, and visual agnosia., Objective: To define the cognitive profile of PCA and compare to the typical, primary amnestic dementia of the Alzheimer's type (DAT)., Methods: The authors used standard cognitive tests and a novel battery designed to reflect dysfunction in both ventral (Object, Face & Color Agnosia Screen [OFCAS]) and dorsal (complex pictures and compound stimuli) visual streams. The authors identified 19 patients with PCA and compared their performance to a matched group of patients with DAT and normal controls., Results: Patients with PCA were younger with marked impairment in visuospatial tasks, reading, and writing but relative preservation of memory compared to DAT using standard tests. Dorsal stream signs were most prevalent among the patients with PCA with no pure ventral stream syndromes found. All novel tests distinguished reliably between subjects with complex picture descriptions and processing of compound stimuli showing the most significant differences compared to DAT., Conclusions: PCA is predominantly a dorsal stream syndrome, distinct from typical DAT, which involves occipitotemporal regions over time.

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.

Background: In vivo imaging of the dopamine transporter with [99mTc]TRODAT-1 (TRODAT) and olfactory testing have both been proposed as potential biomarkers in Parkinson disease (PD)., Objective: To evaluate the relationship between TRODAT SPECT imaging, odor identification skills, and motor function in patients with early PD., Methods: Twenty-four patients with a clinical diagnosis of early-stage PD (mean Hoehn & Yahr stage = 1.4) underwent TRODAT imaging, Unified PD Rating Scale (UPDRS) ratings of motor function, and administration of the University of Pennsylvania Smell Identification Test (UPSIT). Brain images were obtained using a standardized processing protocol and specific uptake ratios for striatal regions of interest were calculated. Partial correlations between the imaging indices, disease duration, UPSIT scores, and UPDRS motor scores were then calculated., Results: UPSIT scores were correlated with TRODAT uptake in the striatum as a whole (r = 0.66, p = 0.001). The putamen showed the strongest correlation with the UPSIT (r = 0.74; p < 0.001). The correlation between dopamine transporter density in the caudate and UPSIT was moderate (r = 0.36, p = 0.11), but was not significant., Conclusions: Olfactory function is highly correlated with dopamine transporter imaging abnormalities in early Parkinson disease (PD). Further studies are warranted to determine whether changes over time in these two measures are also correlated in early PD.

Objective: To develop a quantitative method of assessing cognitive anosognosia in six cognitive and two noncognitive domains., Methods: Control (n = 32) and probable Alzheimer disease (pAD) (n = 14) subjects self-estimated memory, attention, generative behavior, naming, visuospatial skill, limb praxis, mood, and uncorrected vision, both before and after these abilities were assessed. Based on this estimate and their performance the authors calculated an anosognosia ratio (AR) by dividing the difference between estimated and actual performance by an estimated and actual performance sum. With perfect awareness, AR = 0. Overestimating abilities would yield a positive AR (< or =1); underestimation would yield a negative AR (> or =-1)., Results: Relative to controls, pAD subjects demonstrated anosognosia. Pre-testing (off-line), pAD subjects overestimated their visuospatial skill; post-testing (on-line), pAD subjects overestimated their memory. Control subjects also made self-rating errors, underestimating their attention pre-testing and overestimating limb praxis and vision post-testing., Conclusions: This anosognosia assessment method may allow more detailed examination of distorted self-awareness. These results suggest that screening for anosognosia in probable Alzheimer disease (pAD) should include self-estimates of visuospatial function, and that, in pAD, it may be useful to assess anosognosia for amnesia both before and after memory testing.

Objective: To report the assessment of a patient exhibiting gustatory agnosia., Methods: Preoperative and postoperative neuropsychological, neuroimaging, and chemosensory evaluations were performed in a 39-year-old woman undergoing surgical treatment for intractable epilepsy., Results: Preoperative MRIs showed bilateral (right > left) atrophy in the medial temporal lobes and complete atrophy of the left insula. Evaluation of gustatory function revealed normal suprathreshold intensity estimation, affective evaluation, and detection thresholds but elevated recognition thresholds. A functional neuroimaging study showed activation to stimulation of aversive taste in the left amygdala. Surgical treatment entailed resection from the left medial temporal lobe that included the region of amygdala that had responded to taste. Postoperatively, detection, naming, and intensity estimation for taste remained normal, but the patient was unable to recognize different tastes (sweet, sour, salty, and bitter). A second evaluation 2.5 years after her surgery revealed no change in taste ability., Conclusion: The anteromedial temporal lobe has an important role in recognizing taste quality.

The authors describe a patient with auditory agnosia caused by a tectal germinoma. Despite having normal audiometric tests, the patient failed to recognize words and musical characters. On head MRI, the inferior colliculi were infiltrated by tumor. Neuropsychological tests revealed severe impairment in recognition of environmental sounds and words, defective musical perception, and stop consonant-vowel discrimination. Inferior colliculus may play a role in the analysis of sound properties.

Objective: To characterize the clinical course of pathologically diagnosed hippocampal sclerosis dementia (HSD)., Background: Dementia associated with HSD is incompletely characterized. Previous studies suggest similarities to both Alzheimer disease (AD) and frontotemporal dementia (FTD)., Methods: Case-control analysis of the clinical course of patients with HSD, FTD, and AD from a neuropathology autopsy series conducted by a university hospital. Case histories were reviewed. Cumulative prevalence of behavioral, cognitive, psychiatric, and language symptoms were compared between groups, as was time of symptom onset. Clinical diagnostic criteria for FTD and AD were applied to case histories. Sensitivity and specificity of clinical FTD diagnostic criteria (Report of the Work Group on FTD and Pick's disease) were computed., Results: Cumulative prevalence of symptoms in HSD was most similar to that of FTD and differed from AD. Behavioral abnormalities such as decreased grooming and inappropriate behavior were more prevalent in HSD and FTD than AD. Hyperorality, inappropriate behavior, and decreased interest had earlier onset in HSD and FTD. Cognitive symptoms of disorientation, dyscalculia, apraxia, and agnosia were more prevalent in AD, as were psychiatric symptoms of hallucinations, delusions, and aggression. Most HSD patients met diagnostic criteria for FTD. Criteria sensitivity was 64.0% and specificity was 73.7%., Conclusions: FTD is a clinical syndrome associated with heterogeneous neuropathology. The clinical course of HSD is more similar to that of FTD than AD. These findings, together with the neuropathologic data presented in the accompanying article, support expanding the scope of FTD (Pick complex) to include HSD.

Background: Face imagery can access facial memories without the use of perceptual stimuli. Current data on the relation of imagery to the perceptual function and neuroanatomy of prosopagnosic patients are mixed, and little is known about the type of facial information patients can access through imagery., Objective: The authors wished to determine 1) which lesions abolished face imagery in prosopagnosia, 2) if deficits in perceiving facial structure were paralleled by similar deficits in imagery, and 3) if covert recognition of faces correlated with the degree of residual imagery for faces., Methods: The authors tested nine prosopagnosic patients who had been tested previously for perception of facial configuration and covert recognition of famous faces. The authors constructed a battery of 37 questions that asked subjects to imagine the faces of two celebrities and to choose which one had a certain facial property. Half were questions about facial features and half were about overall facial shape., Results: Imagery was abolished only by anterior temporal lesions. Imagery for facial shape but not features was degraded by lesions of the right hemisphere's fusiform face area, which severely impaired perception of facial configuration. Feature imagery was degraded only when there was associated left occipito-temporal damage. Covert recognition was found when either configural perception or imagery was severely damaged, but not when both were abnormal. In patients with impaired configural perception, covert recognition correlated with feature imagery, suggesting that feature-based processing may drive residual covert abilities in these patients., Conclusion: Although anterior temporal cortex may be the site of facial memory stores, these data also support hypotheses that perceptual areas like the fusiform face area have parallel contributions to mental imagery. The data on covert recognition are consistent with a view that it is the residue of a partially damaged face-recognition network. Covert recognition may reflect the degree of damage across components of a network rather than mark a specific form of prosopagnosia or a dissociated pathway.

To test whether low-frequency repetitive transcranial magnetic stimulation (rTMS) of sensorimotor cortex (SM1) has prolonged effects on somatosensory function, eight subjects were given 900 TMS pulses over the left hand SM1 (0.9Hz, 90% of the resting motor threshold) or at sites 3 cm anterior or posterior to it. Tactile threshold of the right hand was increased for a short duration after rTMS over SM1, but two-point discrimination and median nerve SEPs were unaffected after rTMS at any sites.

Background: Unilateral spatial neglect has been rarely reported in patients with AD, although they often have right and left asymmetry of temporoparietal dysfunction., Objective: To investigate if patients with AD would show unilateral spatial neglect in the line bisection test, and to reveal the relationship between their neglect and the area of cerebral dysfunction., Method: Thirty-two patients with mild to moderate AD and 32 age-matched healthy control subjects underwent an extensive line bisection test. SPECT was also obtained for the patients., Results: Rightward bisection errors exceeded the normal range in 25% of patients with AD. They exhibited greater rightward errors for the longer lines in the left hemispace than in the right hemispace, and with the right hand than with the left hand; this corresponds to the characteristics of neglect seen after right hemisphere lesions. All patients who bisected 200 mm lines with errors over 10 mm showed disproportionate lowering of performance IQ and asymmetric right hemisphere hypoperfusion, especially in the temporoparietal region. Seventy-five percent of the patients performed normally in the center presentation but erred slightly toward the body midline in the right and left hemispaces., Conclusion: Left unilateral spatial neglect in mild to moderate AD may be rather common if tested with the line bisection test. Rightward errors over 10 mm suggest right temporoparietal dysfunction. In AD, three or more bisections of 200 mm lines in the center presentation are recommended for detection of neglect. Patients with AD but without neglect may have difficulty in shifting attention into the peripheral sector of the egocentric space.

The cause of anosognosia for hemiplegia (AHP) remains unclear. Weakness is detected when there is a mismatch between the expectancy of movement and the sensory perception of movement. The feed-forward hypothesis of AHP posits that there is a failure of detection because there is a loss of motor intention and expectancy of movement. We tested motor intention by measuring the activation of proximal muscles (pectoralis majoris) while subjects squeezed a dynamometer with each hand. We tested a group of normal controls, a group of patients with hemiparesis, a patient with neglect, a patient with resolved AHP, and a patient with persistent AHP. The patient with AHP did not contract either of his pectoralis muscles when asked to squeeze with his contralesional, paretic hand, yet he contracted both of them when squeezing the dynamometer with his ipsilesional hand. Normal controls, hemiparetic controls, and the patient with hemispatial neglect contracted both pectorales when asked to squeeze with each hand. The pattern of activation seen in the patients with persistent AHP and resolved AHP demonstrates a loss of motor intention and lends support to the feed-forward hypothesis of AHP.

We compared patients with unawareness of hemiplegia lasting more than 1 month after right hemisphere stroke with other patients with right hemisphere stroke who became aware of hemiplegia within a few days after onset. Patients with persistent unawareness invariably had severe left hemisensory loss and usually had severe left spatial neglect. They were almost always apathetic; their thought lacked direction, clarity, and flexibility, and they had at least moderate impairment of intellect and memory. Their right hemisphere strokes were large and always affected the central gyri or their thalamic connections and capsular pathways. In addition, there was evidence of at least mild left hemisphere damage, most commonly caused by age-associated atrophy. The pathogenesis of anosognosia for hemiplegia may involve failure to discover paralysis because proprioceptive mechanisms that ordinarily inform an individual about the position and movement of limbs are damaged, and the patient, because of additional cognitive defects, lacks the capacity to make the necessary observations and inferences to diagnose the paralysis. We discuss the implications of this "discovery" theory and contrast it with other explanations of anosognosia.

We measured the severity of left spatial neglect in 29 patients, 2 to 4 weeks after right cerebral infarction. The severity of neglect increased with both the size of the lesion and the degree of premorbid diffuse cortical atrophy. Recovery over 3 to 5 months from an initially moderate to severe neglect was less complete in patients with cortical atrophy. The importance of lesion size shows that spared areas of the right hemisphere limit the severity and duration of left neglect. The importance of premorbid atrophy suggests that the integrity of the left hemisphere is also essential.

A left-handed patient was studied who had the acute onset of alexia without agraphia, a left homonymous hemianopia, and prosopagnosia. Neurodiagnostic tests including computerized axial tomography and angiography disclosed bilateral lesions in the posterior cerebral hemispheres involving the splenium of the corpus callosum. Neuropsychologic examination indicated marked impairment of facial discrimination, deficient naming and memory of presented visual material, and mild visual agnosia with relative preservation of other cognitive functions. These findings are consistent with a double disconnection syndrome involving disconnection of the dominant angular gyrus and right inferotemporal cortex from their bilateral visual inputs.

In pure word deafness, comprehension and repetition of speech are impaired, but reading, writing, and spontaneous speech are preserved. Pure word deafness is distinguished from generalized auditory agnosia by the preserved ability to recognize environmental sounds. We examined a patient with pure word deafness associated with bilateral infarctions of the primary auditory cortex, who could use auditory affective intonation to enhance comprehension. The primary auditory cortex seems to be essential for comprehending speech, but comprehension of nonverbal sounds and affective prosody may be mediated by other cerebral structures such as the auditory association cortex.

Agnosia is an abnormality of recognition that is not explained by sensory or cognitive disorders. We studied a patient who had combined visual-tactile agnosia without prosopagnosia after a left hemisphere infarct. Although he copied figures presented visually and he performed intramodal or crossmodal visual-tactile matches, he could not indicate recognition by either sight or touch. The lesion involved areas of the occipital and temporal lobes that may be important for human performance of tasks that require construction and application of meaning to percepts presented both visually and tactually.