Smoking and Psoriasis as Synergistic Risk Factors in Periodontal disease.

In the literature both smoking and psoriasis are discussed as predisposing factors for chronic periodontal disease. It is also known that smoking leads to deterioration in both psoriasis and periodontal disease. However, up to now, the only study to address the question what effect the co-occurrence of psoriasis and smoking has on the periodontal status of the individual, was a previous study of ours. In the present study, we repeated our measurements in an extended sample. 82 psoriatic patients and 117 controls participated, who all received a full-mouth examination so that their periodontal status could be determined. The analysis was aimed at finding out about to what extent the individual risk factors (i.e. smoking and psoriasis) increased the chance of the occurrence of the advanced stages of periodontal disease. The odds ratio for smoking was 1,32 (p = 0,465), and 1,85 for psoriasis (p = 0,163). In those patients who smoked, the odds ratio was 6,22 (p < 0,001), which is three times higher than the simple combination of odds. This suggests that the risk factors are in a synergistic relationship.

Progressive supranuclear palsy in a family with TDP-43 pathology.

UNLABELLED: A member of a family with an autosomal dominant pattern of frontotemporal dementia (FTD) with a TDP-43 pathological substrate in other members and no mutations in FTD-associated genes developed behavioral variant FTD followed by Progressive Supranuclear Palsy. Autopsy revealed a pure tauopathy of PSP pattern. CONCLUSIONS: The findings raise the possibility of shared pathogenic pathways and a proximal genetic abnormality between PSP and FTLD-43.

Galantamine in frontotemporal dementia and primary progressive aphasia.

BACKGROUND/AIMS: The treatment of frontotemporal dementia (FTD) has been mainly symptomatic. Small randomized or open-label case control studies of neurotransmitters have been inconclusive. We tried galantamine in the 2 most common varieties of FTD. METHOD: Thirty-six behavioral variety FTD and primary progressive aphasia (PPA) patients were treated in an open-label period of 18 weeks and a randomized, placebo-controlled phase for 8 weeks with galantamine. The primary efficacy measures were the Frontal Behavioral Inventory, the Aphasia Quotient of the Western Aphasia Battery, the Clinical Global Impression of Severity and the Clinical Global Impression of Improvement. RESULTS: No significant differences in behavior or language were found for the total group. A treatment effect (p = 0.009), in a subgroup of subjects with PPA in the global severity score, in favor of galantamine was detected in the placebo-controlled withdrawal phase but was not considered significant after correction for multiple comparisons. The language scores for the treated PPA group also remained stable compared to the placebo group, which showed deterioration. CONCLUSION: Galantamine is not effective in the behavioral variety of FTD, but a trend of efficacy is shown in the aphasic subgroup, which may be clinically significant. Galantamine appeared safe in FTD/PPA.

Neuropsychological deficits in vascular dementia vs Alzheimer's disease. Frontal lobe deficits prominent in vascular dementia.

OBJECTIVE: To detect neuropsychological differences between Alzheimer's disease (AD) and vascular dementia (VAD). DESIGN: Neuropsychological measures were compared in clinically defined AD and VAD patient groups. SETTING: Ambulatory and hospitalized patients were referred to a behavioral neurology clinic and to the neuropsychology department of a teaching hospital. PATIENTS: Consecutive, referred patients who fulfilled National Institute of Neurological Disorders and Stroke/Alzheimer's Disease and Related Disorders Association and Diagnostic and Statistical Manual of Mental Disorders, Revised Third Edition, criteria for AD and VAD were selected to participate in the study based on the history and clinical findings. A modified ischemic score of 3 or less was an independent selection criterion for AD (n = 103) and a score of 4 or more for VAD (n = 25). Computed tomography or magnetic resonance imaging was used to exclude other structural causes. Patients with cognitive changes related directly to a stroke were excluded. Patients were matched for age, education, age at onset, and severity of dementia. MEASURES: The variable measures were the subtests of the Wechsler Adult Intelligence Scale-Revised (WAIS-R), Wechsler Memory Scale-Revised, Mattis Dementia Rating Scale (MDRS), and Western Aphasia Battery (WAB). Patients were further stratified into mild and severe dementia categories, based on their performance on the MDRS. RESULTS: Variables that were significantly different were selected for discriminant function analysis. The Writing subtest of the WAB, the Picture Arrangement subtest of the WAIS-R, and the Motor Performance subtest of the MDRS were the best discriminators of AD and VAD in the overall and severely affected populations. Patients with VAD performed significantly worse on the MDRS Motor Performance subtest, the WAIS-R Picture Arrangement subtest, the WAB Writing subtest, the WAIS-R Object Assembly subtest, and the WAB Block Design subtest. The AD group performed significantly worse on the WAB Repetition subtest, and patients with severe AD performed significantly worse on the Story Recall test. CONCLUSIONS: Patients with VAD performed worse on tests that are influenced by frontal and subcortical mechanisms. Patients with AD performed worse on memory and some language subtests.

Arnold Pick and German neuropsychiatry in Prague.

Circumscribed focal atrophy with frontal lobe dementia and progressive aphasia, as described originally by Arnold Pick, has been recognized recently as being much more common than previously believed. Although Pick disease became linked with argyrophilic inclusions (Pick bodies) and swollen neurons (Pick cells), the majority of focal atrophies have findings that are a variation of the classic histologic features. We discuss Pick's background and the circumstances that led to his major contributions to the study of behavioral neurology. We also review his original articles, the articles that subsequently established the entity of Pick disease, and historical documents pertaining to the continuation of German-language education in Prague after Prague's independence from the Austro-Hungarian monarchy. Arnold Pick's life and career exemplify the integration of neurology, psychiatry, and neuropathology, which represents one of the major contributions of German neuropsychiatry to the study of the nervous system. Pick is a major intellectual ancestor of present-day neurology.

On drawing impairment in Alzheimer's disease.

Spontaneous drawings of 38 patients, diagnosed by the National Institute of Neurological Disorders and Stroke-Alzheimer's Disease and Related Disorders Association criteria as "probable Alzheimer's disease," and of 39 normal control subjects were analyzed by two independent observers using a standardized scoring system. Drawings of patients with Alzheimer's disease displayed fewer angles, impaired perspective and spatial relations, simplification, and overall impairment compared with those of the control subjects. This represents a combination of the deficits seen following right- and left-hemisphere lesions. Neglect, tremor, and perseveration were not prominent. Drawing impairment was relatively independent of language or memory impairment, but drawing performance was related to perceptual and executive dysfunction in the visuospatial domain. Deterioration was followed up for up to 3 years.

Profiles of language impairment in primary progressive aphasia.

OBJECTIVE: The profile of language impairment in patients with primary progressive aphasia in comparison with the language impairment in patients with Alzheimer's disease and after stroke. DESIGN: The Western Aphasia Battery and the Mattis Dementia Rating Scale evaluated the language and cognitive impairment. Follow-up studies were done 1 to 5 years after the initial testing in seven of 10 patients with primary progressive aphasia, and a postmortem examination of the brain was done in two patients. SETTING: All 10 patients with primary progressive aphasia were previously healthy, community-dwelling persons when first tested. PATIENTS AND OTHER PARTICIPANTS: All patients with primary progressive aphasia reported at least a 2-year history of slowly progressive aphasia without other signs of global dementia. The initial Western Aphasia Battery results of the 10 patients with primary progressive aphasia were compared with those of a sample of 10 patients with probable Alzheimer's disease and with those of a sample of 10 patients with aphasia due to a left hemispheric stroke. Both reference samples were matched for age and sex; the sample with stroke-caused aphasia was additionally matched for the aphasia type. RESULTS: Expressive language disability with reduced speech fluency and anomia but preserved language comprehension and nonverbal cognition were typical features in early stages of primary progressive aphasia. Spontaneous speech was significantly more impaired in patients with primary progressive aphasia in comparison with those with aphasia after left hemisphere stroke and with language impairment in patients with Alzheimer's disease. Follow-up examinations revealed continuous, often rapid deterioration of language impairment. The neuropathological examination showed Alzheimer's disease in one patient and Pick's disease in the other. CONCLUSION: The profile of aphasia suggests that primary progressive aphasia tends to affect anterior parts of the language-dominant cortex first.

Localization in transcortical sensory aphasia.

Transcortical sensory aphasia is a syndrome characterized by poor comprehension but excellent repetition. The lesions shown on computed tomography and isotope scans of 15 patients who satisfied the objective criteria based on test scores were studied. The overlap technique showed a unique posterior parieto-occipital location of lesions. The lesions seem to separate into two groups: one is more medial, inferior, and posterior and is clearly in the posterior cerebral artery territory, and the other is relatively more lateral, superior, and anterior and seems to be in a watershed area between middle cerebral and posterior cerebral arteries. The lesion sizes correlated with severity. Frequent recovery and good prognosis are associated with this entity. Association with transient visual agnosia and hemianopsia confirmed the anatomical correlation.

Isotope localization of infarcts in aphasia.

Radionucleide localization of infarcts producing aphasia was undertaken in 65 patients with a scan-test and onset-test interval of one months or less. The scans were traced on anatomical templates without knowledge of the aphasias. A phasics were classified by their test scores according to taxonomic criteria, independently from localization. Scans belonging to each clinically distinct group were overlapped "blindly". The results showed distinct areas for Broca's conduction and Wernicke's aphasics along the parasylvian axis of the lateral templates. Lesions of global aphasics covered all these areas, while transcorticals were outside of them. Lesion size and severity of aphasia showed significant correlation. It is concluded that a brief systematic survey of aphasia like ours is useful in predicting the anteroposterior location and often the depth and extent of lesions.

Cognition and white matter changes on magnetic resonance imaging in dementia.

In a prospective magnetic resonance imaging and cognitive study of 38 demented patients and 15 control subjects, 11 of 27 patients with Alzheimer's disease and 8 of 11 patients with vascular dementia had significant periventricular hyperintensities. Memory and language testing in the early investigation of dementia is useful to distinguish patients with or without periventricular hyperintensities on magnetic resonance imaging. Patients without periventricular hyperintensities are worse on memory and conceptualization tests than patients with periventricular hyperintensities, who tend to be worse on comprehension and attention tests. These differences in cognitive pattern are present between patients with different pathogenesis who are otherwise matched for dementia severity. Language and some nonverbal cognitive deficits correlate with the extent of cortical and ventricular atrophy in Alzheimer's disease.

Posterior internal capsule infarction associated with neglect.

A persistent hemispatial neglect developed in a patient following a small infarction of the posterior limb of the right internal capsule. This defect was restricted to tasks requiring spontaneous motor performance on the contralateral hemispace, especially when carried out under visual control. It was not associated with an extinction phenomenon. Verbal cues improved exploratory behavior, suggesting that hemispatial akinesia in this case was due to an intentional disorder. A pulvinar-parietal disconnection, depriving the right parietal cortex from relevant visuospatial information from the contralateral hemispace, was probably the anatomic basis of hemispatial neglect in this case.

Periventricular and subcortical hyperintensities on magnetic resonance imaging. 'Rims, caps, and unidentified bright objects'.

Magnetic resonance imaging hyper-intensities were classified as periventricular rim, caps, and unidentified bright objects (UBOs). These were quantitated in 100 acute stroke and 23 hemorrhage patients and 59 control subjects selected from 590 consecutive scans. The rims, caps, and ventricular size were rated on a scale from 0 to 3 for severity, and the UBOs were counted. The results indicated that the rim is also frequent in control subjects and increases with age. Unidentified bright objects, caps, and severe rims usually signify pathology, occurring much more frequently in patients with strokes and hemorrhages than in control subjects. Hypertension is a significant risk factor in UBOs and caps, but in rims, the incidence of diabetes is higher. The clinical and pathologic significance of these hyperintensities and their relationship to Binswanger's disease, lacunar state, and "état criblé" is discussed.

Wernicke's encephalopathy after gastroplasty for morbid obesity.

A young woman developed Wernicke's encephalopathy after gastroplasty for morbid obesity. Her clinical presentation of nystagmus and ocular gaze palsies, ataxia, and a sensorimotor neuropathy is described. A small number of previously described patients are compared with respect to clinical features, response to treatment, and investigative procedures.

Multiple dimensions of handedness and the human corpus callosum.

Consistency of right-hand preference correlates with callosal isthmus size. Anterior corpus callosum (CC) regions vary as a function of sex and right- versus left-hand preference. We analyzed regional CC anatomy in a sample of right- and left-handed men and women on the basis of both degree ("Consistency") and direction ("Hand") of hand preference scores. Significant Hand x Consistency effects indicated that both are important factors in CC organization. These data also suggested that a single continuum of hand preference and regional CC size may not be applicable across all subjects.

Subcortical neglect: quantitation, anatomy, and recovery.

Fifteen patients with right hemispheric subcortical infarcts localized by CT or MRI were evaluated with a neglect testing battery. Trimodal extinction was present in one case, visual extinction in three, tactile in three, and auditory in six. Although frequent (10 cases), hemispatial neglect was rarely severe (3 cases). Three cases recovered completely. Large posterior internal capsule lesions were associated with more frequent impairment on a cancellation test. The probable mechanism of hemispatial neglect was attentional in one case and intentional in six. Visual and auditory extinction is explained by striatonigral mechanisms. Although subcortical structures participate in attentional and intentional behavior, cortical structures can substitute for their functions.

Triple "E" syndrome: bilateral locked posterior fracture dislocation of the shoulders.

Bilateral locked posterior fracture dislocation of the shoulders is one of the least common injuries of the shoulder, and this injury has been suggested to be pathognomonic of seizures when diagnosed in the absence of trauma. The authors present a case of idiopathic bilateral locked posterior fracture dislocations of the shoulder, along with a review of the medical literature. The authors also present the "triple E syndrome," describing the possible etiologies of this injury: epilepsy (or any convulsive seizure), electrocution, or extreme trauma.

Apraxia and aphasia: the functional-anatomical basis for their dissociation.

We studied the functional and anatomical relationship between aphasia and apraxia in 177 patients with CT evidence of left hemisphere stroke. In six severe aphasics, praxis was spared; these cases were analyzed in detail. One patient had a small temporal lesion with severe Wernicke's aphasia. Large frontoparietal lesions were found in the others. The sparing of praxis suggested bilateral representation of visuokinesthetic motor patterns, and functionally active right parietofrontal connections. Some of these patients had uncommon patterns of skull asymmetries that may have been related to bilateral distribution of function.

The pathology and nosology of primary progressive aphasia.

We present three cases of primary progressive aphasia (PPA) with Pick-variant pathology to support a hypothesis of an underlying nosologic relatedness. Neuropathologic examination demonstrated focal brain atrophy with corresponding neuronal loss and gliosis, accompanied by superficial spongiosis. Specific histologic findings were ballooned neurons (Pick cells) in the atrophic areas, and in two of the cases, Pick bodies. They were immunoreactive for tau. In contrast to classic Pick's disease, there were no Pick bodies in the hippocampus. The intense neurofilament immunoreactivity of the perikarya of the ballooned neurons greatly facilitated their recognition. Based on our cases and a critical review of the literature, we hypothesize that the common underlying pathology of PPA is a variant of Pick's disease. Furthermore, we propose the concept of "Pick complex" to include other neurodegenerative diseases characterized by focal cortical degeneration, such as PPA, frontal lobe dementia, ALS with PPA, and corticonigral and corticobasal ganglionic degenerations.

Motor impersistence: a right-hemisphere syndrome.

We studied motor impersistence with a standardized clinical test of sustained actions, both in patients with acute focal stroke lesions, who were matched for age and size of lesion, and normal controls. Patients with right-sided lesions had significantly more impersistence than did those with left-sided lesions. The most discriminating tests were eye-closure, mouth-opening, tongue-protrusion, and gaze to the left. Right central and frontal lesions seemed to be more responsible for motor impersistence than were posterior or left-sided lesions. Although motor impersistence is sometimes seen with diffuse cerebral disease, it is also a sign of right-hemisphere lesions; the phenomenon is probably related to mechanisms of directed attention that are necessary to sustain motor activity.

The sensitivity and specificity of MRI in stroke.

We compared MRI and CT in a study of 175 patients; 87 infarcts within a week, 40 from 1 to 40 weeks, 25 a year after onset, and 23 hemorrhages, 18 within 2 weeks and 5 in 4 to 8 weeks. Fifty-nine infarcts and eight hemorrhages had sequential scanning. MRI is more sensitive than CT in the early detection of cerebral infarcts. CT is the method of choice to rule out intracerebral bleeding, but MRI is more specific in later stages of hemorrhage. Periventricular hyperintensity is seen more frequently with diabetes than without. Hyperintense white matter patches are often unrelated to clinical events. MRI is useful in following the evolution of strokes and distinguishing acute and chronic infarcts without contrast agents.