Neuropsychological features of rapidly progressive dementia in a patient with an atypical presentation of Creutzfeldt-Jakob Disease.

BACKGROUND: Creutzfeldt-Jakob Disease (CJD) is a degenerative disease of the brain, characterized by rapid and irreversible decline, with dementia, ataxia, myoclonus, and other neurological and neurobehavioral disorders associated with rapidly progressive spongiform encephalopathy. The mode of transmission and basic pathomechanism remain unclear. The clinical picture of CJD is highly diverse, producing a number of variants. MATERIAL AND METHODS: The patient to be described is a 68-year-old Polish female, JR, clinically diagnosed with CJD. The article presents the case history in detail, with particular emphasis on neuropsychological testing, which was initiated when the patient was still lucid and capable of cooperation. The first presenting symptom was agraphia, followed by hemianopsia and other vision disorders, culminating in visual hallucinations. As the progress of the disease accelerated there was rapidly progressive dementia, aphasia developing to organic mutism, myoclonus, hyperkinesia, ultimately loss of all verbal contact or voluntary movement. RESULTS: JR's neuropsychological parameters declined in a period of less than 3 months from near normal to levels characteristic of severe dementia. CONCLUSIONS: The clinical picture here presented is consistent with that of the Heidenhain variant of CJD, with spongiform encephalopathy beginning in the right occipital lobe. Several features of the case remain atypical, however, including the absence of the most common genetic mutation and the patient's long survival after onset.

Disturbances of mental image processing in post-stroke patients with left and right hemisphere damage.

BACKGROUND: The purpose of this article is to point out significant differences in how mental images are processed by post-stroke patients with left and right hemisphere damage. The issues involved are of theoretical importance because of the light shed on the modularity of cerebral functions, especially the imagination, and of clinical importance due to the better understanding of the underlying pathomechanism. MATERIAL AND METHODS: The research involved 82 right-handed patients with a lesion in the left hemisphere (Group L), 82 right-handed patients with a lesion in the right hemisphere (Group R), and, as a control group, 82 patients with musculo-skeletal disorders not affecting the central nervous system (Group C), matched by age and sex. Image processing of complex notions was examined by using selected items from the Simple Neurolinguistic Test. RESULTS: In the control group, the majority of the patients responded to most of the prompts with polymodal associations of various types. In Group L, responses were dominated by isolated elements of the complex situation, while in Group R the associations were mostly verbal (lexical) and highly restricted in scope. CONCLUSIONS: The results indicate that the loss of LH functions interferes with the ability to assemble pieces of polymodal image information into sensible strings, while the loss of RH functions leaves strings to which little information is attached.

The impact of art therapy on the intelligibility of speech in children with cerebral palsy.

Background. Children with cerebral palsy (CP) frequently have dysarthria, which reduces the intelligibility of their speech and can seriously impede their psycho-social adjustment. Traditional "bottom-up" rehabilitation oriented directly on the articulatory apparatus is not always successful in helping these children to speak intelligibly. The authors have developed a program of art therapy with elements of logopedic therapy to model and stimulate perception and expression, including linguistic performance, which has proven in clinical to be a helpful "top-down" approach to helping these children to speak more early.
Material and methods. Out of the population of CP children treated in the Cracow Rehabilitation Center's Art Therapy Workshop in the period 1994-2001, we identified 14 children, 9 boys and 5 girls, average age 15,3, who showed severe dysarthria at admission without other language or cognitive disturbances. Our retrospective study was based on recorder results from the Auditory Dysarthria Scale and standard neuropsychological tests for fluency of speech, administered routinely once each month during the 4-month art therapy program.
Results. All the children in the study group showed some degree of improvement after art therapy in all tested parameters. On the Auditory Dysarthria Scale, the largest improvements were noted in overall intelligibility (p < 0,0001); less improvement, though still statistically significant (p < 0,001) took place in respect to volume, tempo, and control of pauses. The least improvement was noted in the pronunciation of vowels and consonants (the most purely motor of the measured parameters). All the children also exhibited significant improvement in fluency of speech.
Conclusions. Art therapy with elements of logopedic therapy improves the ability of children with cerebral palsy to engage in purposeful behavior, including speech and language functions, especially intelligibility.

Post-coma paraschizophrenia and quality of life in patients with closed-head injuries.

Background. Organic brain damage is traditionally an excluding criteria for a psychiatric diagnosis of mental illness. In fact, however, the neurobehavioral disturbances exhibited by many CHI patients, especially those who have been comatose for more than a month, are strikingly similar to the symptoms of Type II schizophrenia. The authors propose to use the term "post-coma paraschizophrenia" to describe these disturbances and discuss their negative impact on the quality of life of CHI patients.
Material and methods. The experimental group (Group CHI) consisted of 15 CHI patients, 7 males and 8 females, ranging in age from 17 to 58, treated in the Department of Medical Rehabilitation at the Cracow Rehabilitation Center and the Rehabilitation Clinic at the Bydgoszcz Medical University. All these patients had incurred a closed-head injury resulting in a coma lasting at least one month. He control group (Group SCHI) consisted of 15 patients diagnosed with Type II schizophrenia, matched by age, sex, and level of education to the patients in Group CHI, under treatment in the Psychiatry Department at the Wroclaw Medical University and the Bydgoszcz Medical University. The data reported were based on clinical observation, patient and family interviews, standard neuropsychological tests, the Frontal Behavioral Inventory, and the Quality of Life Scale for Patients with Traumatic Brain Injuries.
Results. Standard neuropsychological tests showed similar dysfunctions in general intelligence and memory, with a tendency in both groups to lower scores in non-verbal parameters. Some interesting qualitative differences in performance are presented. All the CHI patients showed significant signs of frontal syndrome, with a profile resembling fronto-temporal dementia, while the test results from the SCHI patients were more similar to those associated with severe clinical depression. All of the patients in the CHI group exhibited at least some of the symptoms traditionally regarded as indicative of schizophrenia. Positive (formative) symptoms of schizophrenia were found much more often in the patients from Group SCHI, whereas the percentages for the occurrence of particular negative (defective) symptoms are quite similar in both groups.
Conclusions. Patients with closed-head injuries may present with quasi-psychotic symptoms, here termed "post-coma paraschizophrenia". Among the most significant common features of the two syndromes are disorganized behavior, apathy, and disturbances of executive functions. Post-coma paraschizophrenia, which mostly remains untreated because of the lack of neuropsychiatric diagnosis, has a significant negative on the quality of life of CHI patients, and should receive further attention in both theoretical research and clinical practice.

[Pathomechanism and clinical presentation of neurobehavioral disturbances in a patient with MELAS syndrome]. / Patomechanizm i obraz kliniczny zaburzen neurobehawioralnych u pacjentki z zespolem MELAS.

The authors present the results of a longitudinal study of the neurobehavioral disturbances seen in K.S., a 22-year-old female patient with a mitochondrial cytopathy (MELAS) caused by the novel mutation C8293T. K.S. became ill in 1994 at the age of 16. She was referred for diagnosis to several different clinics. Four years after onset, the clinical diagnosis was established in the Department of Medical Rehabilitation at the Cracow Rehabilitation Center; the diagnosis was not confirmed until six years after onset, following the discovery of the mutation in the patient's mtDNA at Columbia University. Since 1996 the patient has presented with progressive dementia and periodic stroke-like episodes that produced fluctuating neurological symptoms. The essential pathomechanism of the neurobehavioral disturbances consists in the fragmentation of complex cerebral processes into their constituent elements; individual functions are frequently correctly executed on a lower level of cerebral organization, but the patient is unable to combine them into a sensible whole. The authors discuss the theoretical and clinical significance of the results presented here.

An evaluation of the effectiveness of patient - physician communication following traumatic brain injury.

Despite the enormous importance of patent-physician communication for treatment and rehabilitation, the topic of interpersonal communication does not find a place in the curriculum of most medical schools. Medical practice in this respect is dominated by improvised solutions emerging from the individual physician's personal communication style, which in many cases leads to breakdowns in communication with patients. The purpose of this article is to describe the general principles governing patient communication and the problems encountered in this regard. Communication is discussed as a social process, which on the one hand runs its course in accordance with certain generally binding rules, and on the other hand is governed by the purposes (both open and hidden) and inclinations of the persons involved in the conversation. Several key concepts in communication are explained, including sender and receiver; text, subtext, and context; verbal and non-verbal communication. A simplified model of the communication process is presented. The research material consisted of a group of 36 TBI patients who underwent treatment at the various centers represented by the authors. The research results are presented on the basis of the assumptions described earlier as pertains to the effectiveness of physicians communication with patients. An example conversation is presented, involving two physicians and a patient, recorded as party of the research project. In order to illustrate the basic errors committed by the various speakers for the needs of specialist in orthopedics, traumatology and rehabilitation, the authors have discussed this conversation according to the qualitative method known as "conversation analysis". Patient drawings are also presented, illustrating their experiences in communication with physicians, along with an in-depth analysis of the messages found in these drawings, using the authors' own methodology of qualitative analysis of patient's self-expression. The results obtained points out some of the causes and effects of communication problems, including the divergent goals of physician and patient, the lack of sensitivity to the context and to the particular problems faced by the other parties involved in the conversation, and physicians' failure to control subtext and non-verbal signals. The conclusions presented by the authors emphasize the complexity of the problems researched here and the necessity to take steps to remedy them.

An evaluation of neuropsychological rehabilitation for patients with high spinal cord injuries and brain damage.

This article describes a program of neuropsychological rehabilitation for high cervical SCI patients with psycho-organic syndrome. Traumatic injury to the cervical spine is frequently accompanied by organic damage to the brain, either caused by the same accident that injured the spine or resulting from secondary causes. The result of concomitant brain damage in cervical SCI patients is psycho-organic syndrome, which consists of both cognitive (higher level language functioning, disturbances in memory, language, attention, and thinking) and personality components (mood and behavioral disorders). Existing rehabilitation programs designed either for tetraplegic patients with no neuropsychological impairment, or for brain-damaged patients without tetraplegia, are not suitable for the problems faced by these patients. Accordingly, the authors have developed a Program of Neuropsychological Rehabilitation as a module added to comprehensive rehabilitation for SCI patients. This program was tested in a natural clinical experiment involving 35 SCI patients with tetraplegia and concomitant brain damage undergoing rehabilitation at the Cracow Rehabilitation Center and the Marian Weiss Rehabilitation Center in Konstancin, Poland. The results of the experiment demonstrate that the neuropsychological rehabilitation program is effective in reducing the symptoms of psycho-organic syndrome. The authors discuss the theoretical and clinical implications of the results for the rehabilitation of patients with cervical spine injuries and concomitant brain damage.

Disturbances of cognitive and emotional functions in patients with whiplash injuries.

The goal of this article is to present the results of research on cognitive and emotional disturbances in patients with the "whiplash" injuries typically incurred as a result of rear-end collisions. Despite considerable controversy and discussion surrounding the whiplash syndrome the most recent literature on the subject contains several reports suggesting that many of these patients complain of specific symptoms that are not only physical, but also cognitive, emotional, and even linguistic, the existence of which can be detected by standard neuropsychological tests. In their own research involving 58 patients with late "whiplash" syndrome and an equal number of normals, the authors have confirmed the occurrence of objective symptoms in various tests. The discussion considers the etiology of these symptoms in various tests. The discussion considers the etiology of these symptoms and the significance of the results for rehabilitation.

The rehabilitation of executive functions in patients with closed-head injuries.

One of the most difficult problems in the rehabilitation of patients with closed-head injuries is posed by executive function disturbances, that is, difficulty in making the transition from thinking to acting. This article presents the working hypotheses and a description of a rehabilitation program developed by the authors, oriented directly towards training executive functions, in contract to the existing standard program, based on physical therapy with the addition of neuropsychological exercises directed towards training particular cognitive functions. The program consists of exercises supporting the development of not only cognitive and emotional-motivational elements, but also mediating metaprocesses and behavioral processes. In their own research involving 24 patients the authors confirmed the existence of objective disturbances of executive functioning in all subjects. The patients were divided into two groups: a control group of 12 patients treated with the standard program, and an experimental group of 12 patients who received the new rehabilitation program. The results obtained clearly indicate the superior effectiveness of the new program, not only in terms of executive functions per se, but also in transfer to other neurosychological parameters not treated separately. The authors discuss the etiology of executive function disturbances and the significance of the results obtained for rehabilitation. The authors' Clinical Test of Executive Functions is also presented.