Severe Frontal Lobe Research Articles

Assessment of anosognosia in persons with frontal lobe damage: Clinical utility of the Mayo-Portland adaptability inventory (MPAI)

Objectives: To determine if the Mayo-Portland Adaptability Inventory (MPAI) demonstrates clinical utility in differentiating between persons with severe TBI and frontal lobe damage/anosognosia and persons with mild TBI and no frontal lobe damage.Participants: Forty-three persons with TBI and documented frontal lobe damage (mean age = 34; mean time since injury = 5.2 years) and 69 persons with mild TBI and no frontal lobe damage (mean age = 34.3; mean time since injury = 4.8 years).Measure: MPAI.Results: Total inventory and select sub-category difference scores were significantly greater in the frontal lobe group than in the non-frontal lobe group. However, as expected, there was no significant difference between the two groups on the mobility sub-category difference scores.Conclusions: The MPAI appears to be potentially clinically useful in assessing for frontal lobe damage and associated anosognosia in patients with TBI.

Successful closure of recurrent traumatic csf rhinorrhea using the free rectus abdominis muscle flap

BACKGROUND We present two patients in whom a free rectus abdominis muscle flap was used to close recurrent traumatic CSF rhinorrhea. CASE DESCRIPTION CT scan of both patients showed frontal lobe atrophy and porencephaly after contusional hematoma. In the first patient, because the site of CSF leakage was not identified and the patient underwent three unsuccessful attempts to close the fistula using the fascia lata, we treated the patient by unifying all paranasal sinuses and by filling them with a free rectus abdominis muscle flap. In the second patient, CSF rhinorrhea recurred 6 years after closure of the fistula using the fascia lata. The patient underwent separation of a porencephalic cyst from the paranasal sinus and a free muscle flap was placed extradurally, because the CSF pulse pressure in the enlarged left anterior horn eroded the previously repaired fascia lata, resulting in the recurrence of CSF leakage. CONCLUSION Although duraplasty is the primary procedure for repairing dural fistulas, the vascularized free muscle flap is an alternative method when the location of the fistula is not identified or the patient with recurrent CSF rhinorrhea has severe frontal lobe atrophy and porencephaly.

Episodic memory, semantic memory, and amnesia.

Episodic memory and semantic memory are two types of declarative memory. There have been two principal views about how this distinction might be reflected in the organization of memory functions in the brain. One view, that episodic memory and semantic memory are both dependent on the integrity of medial temporal lobe and midline diencephalic structures, predicts that amnesic patients with medial temporal lobe/diencephalic damage should be proportionately impaired in both episodic and semantic memory. An alternative view is that the capacity for semantic memory is spared, or partially spared, in amnesia relative to episodic memory ability. This article reviews two kinds of relevant data: 1) case studies where amnesia has occurred early in childhood, before much of an individual's semantic knowledge has been acquired, and 2) experimental studies with amnesic patients of fact and event learning, remembering and knowing, and remote memory. The data provide no compelling support for the view that episodic and semantic memory are affected differently in medial temporal lobe/diencephalic amnesia. However, episodic and semantic memory may be dissociable in those amnesic patients who additionally have severe frontal lobe damage.

Mapping of a disease locus for familial rapidly progressive frontotemporal dementia to chromosome 17q12-21.

Familial frontotemporal dementia (FTD) is a complex disorder with lack of distinctive histopathological markers found in other types of dementia. Most of the linkage reports from FTD families map the disease loci to chromosome 17q21-22. However, FTD is genetically heterogeneous, as linkage also has been reported to chromosome 3. In the present study, we investigated the genetics of a Swedish family with an early-onset type of rapidly progressive FTD, associated with muscular rigidity and akinetic movements. Neuropathological features such as severe frontal lobe degeneration, spongy changes, and gliosis were present in affected family members. We here report probable linkage to chromosome 17q12-21 with a maximum two-point lod score of 2.76 at theta = 0 for marker D17S806, and a peak multipoint lod score of 2.86 for the same marker. Linkage to chromosome 3 was excluded, as two-point lod scores of -2.79, and -2.27 at theta = 0.01 for markers D3S1603 and D3S1552, respectively, were obtained. Sequencing of the translated exons of a strong candidate gene in the linked region of chromosome 17, the tau gene, failed to identify any mutations segregating with the disease.

What are the obstacles for an accurate clinical diagnosis of Pick's disease? A clinicopathologic study.

Several studies have evaluated the reliability and validity of the clinical diagnosis of Alzheimer's disease (AD) using well-defined neuropathologic criteria, but none has attempted to evaluate the diagnostic accuracy of Pick's disease. We determined the accuracy of the clinical diagnosis of Pick's by presenting 105 autopsy-confirmed cases of Pick's (n = 7) and related disorders (non-Pick's, n = 98) as clinical vignettes in randomized order to six neurologists who were unaware of the autopsy findings. The group of raters had moderate to fair agreement for the diagnosis of Pick's as measured by the kappa statistics. The sensitivity for the diagnosis of Pick's for the first visit (mean, 53 months after onset) and last visit (mean, 78 months after onset) was low (range, 0 to 71%), but specificity was near-perfect. Median positive predictive values at both visits were 83 to 85%. False-negative misdiagnoses mainly involved AD. False-positive diagnoses were rare and occurred with corticobasal degeneration (first visit) and with dementia with Lewy bodies (last visit). Pick's was also misdiagnosed by primary neurologists. The best clinical predictors for the early diagnosis of Pick's included "frontal" dementia, early "cortical" dementia with severe frontal lobe disturbances, absence of apraxia, and absence of gait disturbance at onset. However, the first neurologic evaluation in some of the Pick's cases took place in advanced stages of the disease. Our findings suggest that this disorder is underdiagnosed in clinical practice. Although the low sensitivity for the clinical diagnosis of Pick's is disappointing, our data suggest that when clinicians suspect Pick's, their diagnosis is almost always correct. Absence of awareness of the main features of this disorder and of specificity of the frontal lobe syndrome may partially explain the low detection of Pick's disease.

Delusional Misidentification in Alzheimer’s Disease: A Summary of Clinical and Biological Aspects

Delusional misidentification symptoms (DMS) are common in Alzheimer's disease (AD) and they are frequent sources of serious distress for patients and particularly caregivers. We observed DMS in around 30% of the patients with moderate to severe AD in two independent prospective studies; the Capgras type, phantom boarder, mirror and TV DMS were found most frequently. Patients with DMS showed increased EEG delta-power over the right hemisphere, their CT scans showed more severe right frontal lobe atrophy, and the number of their pyramidal cells in area CA1 was lower than in the patients without DMS. This may indicate that the development of DMS in AD can be promoted by certain patterns of brain degeneration which affect systems relevant to the recognition and updating of memories, while verbal skills may initially be left largely intact.

Dementia of frontal lobe type: neuropathology and immunohistochemistry.

Brains from 12 patients dying with a clinical diagnosis of frontal lobe dementia have been examined at post mortem. In pathological terms four groups were encountered. Groups A and B showed severe frontal and temporal lobe atrophy characterised histologically in group A by severe neuronal loss, spongiform change of the superficial laminae, and mild astrocytosis; in group B severe neuronal loss was accompanied by intense gliosis but with little or no spongiform change. Two patients in this latter group also showed inclusions in frontal cortex and hippocampus typical of "Pick bodies"; such patients were considered as having classic "Pick's disease". Group C patients showed severe striatal atrophy with variable cortical (frontal or temporal) involvement, with histological changes similar to patients in groups A and B. The single patient in group D showed mild frontotemporal atrophy with spongiform degeneration of the superficial laminae of the cortex and nigral damage, and was considered to have motor neuron disease with dementia. This study is consistent with previous reports showing that the clinical syndrome of frontal lobe dementia is pathologically heterogeneous. However, the nosological relationships within these pathological variants, and between them and conditions such as progressive aphasia were similar histopathological changes are present, remain uncertain.

Heterogeneity in Alzheimer's disease: progression rate segregated by distinct neuropsychological and cerebral metabolic profiles.

In an attempt to define possible subgroups of Alzheimer's disease, 21 patients satisfying current clinical diagnostic criteria for this disorder were divided on the basis of progression rates of symptoms. Thirteen patients with relatively rapid intellectual deterioration did not differ from eight patients showing slow progression with respect to global intellectual performance, sex, or age at onset of symptoms. Neuropsychological testing revealed that although the two groups were indistinguishable in verbal or visuospatial functions associated with the parietotemporal cortex, the more rapidly deteriorating group had significantly greater impairment in executive functions attributed to the frontal lobe. PET scans showed equivalent reductions in glucose metabolism in the parietotemporal cortex, but patients with relatively fast progression had significantly greater hypometabolism frontally. These results suggest an association between relatively severe frontal lobe involvement and a rapid clinical course that might have important implications for the development of treatment strategies for patients with Alzheimer's disease.

Learning and retention of a daily activity schedule in a patient with alcoholic Korsakoff's syndrome

Abstract This study examined the ability of a memory-impaired patient with alcoholic Korsakoff's syndrome to learn and retain a schedule of daily activities. A letter-fragment cueing method was used as a teaching technique. Despite evidence of severe frontal lobe deficits and high levels of anxiety the patient showed a strong learning effect relative to baseline. In addition, considerable retention after termination of training was evident. When the activity schedule was altered, the patient learned the new information without direct training. Results are discussed in terms of the reported rigidity of domain-specific learning, the influence of mood states, and the spontaneous use of memory techniques.

COGNITIVE IMPAIRMENT IN EARLY, UNTREATED PARKINSON'S DISEASE AND ITS RELATIONSHIP TO MOTOR DISABILITY

Current knowledge of cognitive dysfunction in Parkinson's disease (PD) has largely been obtained from studies of chronically treated patients in whom effects of disease chronicity, treatment, depression and dementia are confounding factors. Studies of untreated patients have examined few cognitive domains and relationships between cognition, depression and motor disability have been incompletely explored. Accordingly, we studied 60 consecutive patients with newly diagnosed, untreated, idiopathic PD and 37 matched, healthy control subjects; no subject had clinical dementia or depression. All subjects received tests of specific processes of memory and cognition, including working memory, verbal and non-verbal short- and long-term memory, language, visuospatial capacity, set-formation and shifting and sequencing. Patients also received quantitative global clinical measures of severity of dementia, depression and motor disability. The PD group as a whole showed deficits in immediate recall of verbal material, language production and semantic fluency, set-formation, cognitive sequencing and working memory and visuomotor construction. However, this group was unimpaired in immediate memory span, long-term forgetting, naming, comprehension and visual perception. Language deficits and more severe frontal lobe impairments were confined to those PD patients scoring abnormally on a Mini Mental State examination. Motor disability correlated strongly with severity of depression but weakly with cognitive impairment. Cognitive sequencing, set-formation and set-shifting deficits tended to associate with depression, but otherwise there was no association between cognition and depression. The results indicate dissociation of cognition and motor control in early PD which suggests that cognitive dysfunction is largely independent of frontostriatal dopamine deficiency underlying motor disability. Some, but not all, of the frontal lobe deficits of chronic disease are detectable in early, untreated PD. The pathogenesis of the cognitive deficits shown here appears to involve extrastriatal dopamine systems or non-dopaminergic pathology. Longitudinal study is necessary to determine whether increasing disease duration exacerbates the early cognitive deficits and affects new cognitive domains, in addition to producing increasing motor disability.

Treatment with L-DOPA in two cases of organic brain syndrome.

Summary(1) In both of the two cases associated with organic brain syndrome, which were presumably based on the frontal lobe damages, outstanding clinical improvements were observed by oral administration of L‐DOPA, 200 to 400 mg daily.(2) Case 1 showed marked depressive symptoms, such as emotional weakness, depressive mood, autistic tendency, apathy and lack of spontaneity due to severe frontal lobe damage by an automobile accident. Treatment with L‐DOPA improved his psychic symptoms. He acknowledged his thanks for the L‐DOPA treatment by his words that he felt he had regained his former self. When the drug was withdrawn, psychotic symptoms reappeared.(3) Case 2, who was associated with depressive psychic symptoms after carbon monoxide poisoning and whose pneumoen‐cepholographs indicated the presence of frontal lobe atrophy, showed remarkable elevation in his mood and in his activity level following administration of L‐DOPA. Psychic alterations were most outstanding 14 to 15 days after the commencement of L‐DOPA administration and the degree of elation tended to settle down corresponding to the decrease in the dosage.It can be concluded that L‐DOPA is also potent in some cases of suppressed emotion due to organic brain damages. A decrease in stimulation from damaged frontal lobe projected to noradrenergic system of the hypothalamus may cause hypofunction of the system and subsequently reflect the state of hypotonic affective disturbance. Administration of L‐DOPA may accelerate the synthesis of dopamine function to the normal level.