For years Behavioral Neurology studies of focal syndromes has depended on patients with stroke and tumor lesions. With the development of imaging tools such as voxel based morphometry degenerative brain disease has expanded anatomical related syndromes such as the primary progressive aphasias. This paper describes degenerative brain disease cases illustrating focal syndrome onset related to the anatomy of the initial and most severe degeneration. The paper will start in the frontal area describing a patient with behavioral variant frontotemporal dementia relating the published criteria and different pathologies to this syndrome. The paper then describes a patient with posterior cortical atrophy affecting the occipital and parietal lobes and pointing out that these patients have preserved anterograde memory and other clinical features with which the anatomy correlates. Alzheimer pathology is typical but the syndrome may also be caused by corticobasal syndrome. The third case is one with corticobasal degeneration affecting the parietal lobe which has published criteria and a myriad of pathologies that may cause this syndrome. The next two case illustrations will be semantic dementia from the left more than right temporal lobe and prosopagnosia affecting the right more than left temporal lobe. The paper will discuss the diagnostic criteria and typical pathology of these cases. The paper describes a patient with non-fluent primary progressive aphasia and demonstrates that Progranulin mutation patients may present this way. To round out the causes of primary progressive aphasia the paper will describe a case with logopenic aphasia. The published criteria and typical pathology will be added. Lastly the paper describes an Alzheimer patient that demonstrates different memory pathways with the patient having retained “know how” (procedural memory) but not “know what” (episodic memory) and the underlying pathological anatomy that causes this. In conclusion Behavioral Neurology syndromes have expanded with the study of degenerative dementia.
Behavioral neurology is one of the sub-specialty of neurology that targets the cognitive impairment and behavioral and mental problems of neurological diseases. Neuropsychology is a fundamental tool of behavioral neurology, but behavioral neurology is not a mere paraphrase of neuropsychology. Behavioral neurology includes genetics, pathology, physiology, pharmacology, imaging, rehabilitation, and so on. In this talk, I would like to convey the charm of behavior neurological research reviewing what I have been doing.
Symposium I : The real state of aphasia and strategy of aphasia therapy
To clarify current problem of speech therapy, below three study about management of aphasia in acute stroke were performed. Study 1: epidemiology of aphasia in acute ischemic stroke. Aphasia was observed in 15% of patients with ischemic stroke on admission. Their aphasia improved in 46% of patients, was stable in 44% and worsened in 8% at 10 days after the onset. Aphasia occurred in 2% of patients who had no speech disturbance on admission. Study 2: outcome of aphasic patients. Discharge to home from acute hospital was observed in 9% of ischemic stroke patients with aphasia in 48% without aphasia. Length of hospital stay at rehabilitation hospital was 86 days in patients with aphasia and 64 days in patients without aphasia. Discharge to home from rehabilitation hospital at 3 months after onset is observed in 40% with aphasia and 73% without aphasia. Study 3: Clinical problem in speech therapists. One hundred fifty nine of answers to question about speech therapistʼs clinical problems were classified as therapeutic goal in 32%, symptomatology in 19%, pathophysiology in 16%, education and communication in 8% and consultation to patients in 7%. Conference in their institutes were performed in 44% of them, however anatomical discussion were performed only in 13% of them. It is suggested that speech therapy was performed in short time. Moreover post-graduate education program for speech therapists may be poor.
We tried to propose a procedure by which speech therapy for aphasic patients should be conducted, comparing “the aspect of the disorder” with “the aspect of the improvement” of aphasia. We carried out several factor analyses on data of 267 aphasic patients who were evaluated at the beginning and the end of speech therapy using the Standard Language Test of Aphasia (SLTA) . As for the factor analysis on 26 subtest raw scores of the beginning and the end of speech therapy, we extracted 3 factors. On the other hand, as for the factor analysis on 26 improvement scores (the improvement score was defined as the difference between the subtest score at the beginning and the end of the speech therapy) , we extracted 6 factors. We considered this result meant the structure of the disorder itself and the structure of the improvement of aphasia was different. To compare these structures, we listed the features in the improvement of aphasia, and proposed several important points to conduct intensive speech therapy. Furthermore, we reported a process of speech therapy of single case, and compared the features of improvement with the features of which we listed.
Although aphasia is a common neurological condition, and its diagnostic procedure is mostly established, it is not easy to classify the communicative ability of each patient, as there are huge interindividual differences among patients, and their abilities may vary depending on their circumstances. To evaluate their residual abilities which helps daily communication, it is valuable to observe their abilities in a setting which reflects real social contexts. In this presentation a patient with total aphasia who showed rich communicative ability in a group therapy setting is presented, which was largely discrepant from his results of standardized language evaluation. We found that his residual communicative ability has things much in common with linguistic ability shown to reside in the right hemisphere, and mostly dependent on a particular class of sign classified as ʻindexʼ according to Peirce. The observation also revealed that the group treatment of patients with chronic aphasia provides a unique occasion to participate in social activities which helps to fulfill their psychosocial needs.
In an acute care hospital, it is difficult to conduct the sufficient and through therapy to aphasic patients due to their shortened stay. And recently, speech therapist has no longer required to be spent a lot of time in swallowing-related intervention. I discussed the current situation of the acute aphasia intervention and emphasized the importance of team approach for aphasic patients. As an example of the chronic stage, Ireported about the activity of a local activity support center, which targets aphasic patients, and also results of questionnaire of its members. It was reconfirmed that aphasia therapy needs to aim at the life participation of an aphasic person and social participation as well as improvement of his/her linguistic symptoms.Lastly I mentioned that it is necessary of long-term intervention and continuous support, with the cooperation among acute, recovery and chronic stages. To promote the understanding of aphasia in a society is also crucial.
Symposium II : What is the dorsal visual stream doing ?
Converging evidence indicates that there are three streams of information processing in man: 1) ventral stream, which directed to the temporal lobe for processing shape and color of the object to identify them and to retrieve the knowledge of them; 2) ventro-dorsal stream, which directed to the inferior parietal lobule for processing object location and movement to form conscious representation of objects; 3) dorso-dorsal stream, which directed to the intraparietal sulcus and the superior parietal lobule for processing location movement and shape of the object to control actions toward the object unconsciously. Lesions on the ventro-dorsal stream can produce akinetopsia, or hemispatial neglect. Lesions on the dorso-dorsal stream can produce visuomotor ataxia defective prehension or orienting disability of oneʼs own body. Responsible cites and features of these symptoms are discussed with reference to the functions of those two streams.
It is said that human dorsal stream seems to play a role in processing tool information and reading letters.Continuous Flash Suppression (CFS) is a visual masking technique used for detecting the information processed in the dorsal stream. In CFS, high contrast dynamic random-noise pattern is presented to the dominant eye and low contrast static image is presented to the nondominant eye for 200ms while subjects wore red/green anaglyph glasses. We used this technique to clarify what attributes of tools are processed in the dorsal stream and found that this stream processes elongated shapes but does not process the tool category specifically. Additionally, we are conducting further study to clarify the relationships between dorsal stream and reading function. The current results indicated that some information of letters in right parafoveal vision can be processed in human dorsal stream. CFS would be convenient tool to unveil the function of dorsal stream. However this technique remains some open issues. For example, the cortical information blocked by CFS is unclear and the relationships between this technique and specific brain region. In future, the na ture of CFS should be elucidated using various functional imaging techniques.
Bálint syndrome is a striking set of visuospatial defects displaying all the hallmarks of visuospatial deficits, which are categorized into three physical symptoms: psychic paralysis of gaze, spatial disorder ofattention (dorsal simultaneous agnosia) , and optic ataxia. Most affected people have difficulty in activities of daily living as well as instrumental activities of daily living. Holmes and Inoue also described the similar syndrome, visual disorientation. There is, however, one striking difference between Bálint syndrome and visual disorientation. Bálint explained patientsʼ difficulties in reaching for an object as optic ataxia, that is, visuo-movement incoordination, while Holmes explained it as a visual disturbance in judging distance. We investigated difficulty in judging distance using a patient after right parieto-occipital hemorrhage, who subjectively complained of difficulties in judging distance. We studied ability of judging distance by employing an apparatus (Kowa AS-7JS1) used for acquiring or renewing an oversized vehicle driverʼs license. The performance of the patient was significantly worse than both the normal control and the neglect group.Considering his lesion and the cases in the previous reports, the neural basis for judging distance is considered to be the posterior part of the parieto-occipital lobe; the superior parietal lobule, the posterior part of the inferior parietal lobule, and the cuneus.
Medial parietal region (MPR) has traditionally received little study, since it is rarely lesioned in strokes or accidents. Patients with heading disorientation cannot recognize the direction of their own destination and the positional relationship between two locations in both familiar and novel environments. Therefore, it thought that both egocentric and allocentric representation are affected. The lesions are located in the retrosplenial cortex, posterior cingulate cortex and MPR. Cinical studies, neuroimaging studies and physiological studies suggest that MPR plays a critical role in human navigation, and indicate that MPR has a function to integrate egocentric representation with allocentric representation and, particularly a function to convert allocentric representation into egocentric one.
Impaired distance perception may manifest following brain trauma; however, there are no known reports regarding related intervention methods. We report herein a patient with impaired distance perception and other higher brain dysfunctions due to intracerebal hemorrhage who was followed for 6 years. Although the patient was aware of their various dysfunctions and employed compensatory strategies in order to conduct his daily life, impaired distance perception affected both daily life and participation in hobbies. A ball-throwing approach was designed and implemented as a functional compensatory strategy for impaired distance perception. The patient utilized his retained somatic senses to study a specific distance, and then visually confirmed the same distance in order to relearn how to accurately perceive distances. After approximately 1.5 months, the range of distances they were able to perceive increased, and improvements in daily life were observed. The present findings suggest that functional compensatory strategies may be effective in cases of long-term impaired distance perception.
We reported a case with typing impairment and agraphia without aphasia, alexia, apraxia or construction impairment. The patient was a 69-year-old, right-handed male. Prior to cerebral infarction, the patient was skilled at typing and used the blind-touch typing technique, and his speed of typing was faster than writing.Almost all of his writing errors were errors in direction, length or location of pen-stroke lines, and there were no errors evidential of phonological or meaning disturbance. Such features suggest that his agraphia involved a disturbance in “kinesthetic image of writing.” Similarly, all of his typing errors were due to spatial errors in keying, i.e. miss-touching keys adjacent to the target. There were no errors evidential of phonological disturbances. These features suggest that his typing impairment involved a disturbance in “kinesthetic image of typing.” Two lesions were located: one in the left precentral gyrus, and the other in the left angular expanding to the superior and inferior parietal lobule, not including the posterior portion of the left second frontal gyrus, which is indicated as involved in the phonological process of writing. We consider that highly skilled typing and writing share common neural substrates, and in this case the damage to the region localized for writing simultaneously developed typing impairment.
We reported the efficacy of training for a patient who was considered to have phonological dyslexia. The participant was a 63-year-old male and was right-handed. He showed pseudohomophone effects and several lexicalization errors on non-word reading. Simultaneously, he presented phonological deficits and experienced difficulty in sequential information processing. The training program was consisted of the following: (ⅰ) phonological extraction (extracting the first syllable of a word that was spoken) and phonological blending (blending the last syllables of two words that were spoken) , (ⅱ) phonological blending (blending three syllables of non-words that were spoken one syllable at a time and then quickly repeating the integrated syllables, and (ⅲ) sequential movement of hand shapes (performing a hand action as directed) .The patient was not trained to read aloud while this program was carried out. After three weeks of training, the patientʼs performance in phonological extraction, phonological blending, and sequential movement of hand shapes improved. In addition, the number of non-words that were correctly read significantly increased. However, the patient continued to experience slight difficulty in grapheme-phoneme conversion.His inefficiency in reading non-words might be attributable to his phonological deficit and impaired sequential processing.