1/79. Acquired and isolated asymmetrical palatal palsy.Benign acquired and isolated asymmetrical palatal palsy is a rare condition in childhood. We report on three cases. Typical features include: sudden onset, abnormality of the palatal components of speech (rhinolalia), nasal escape of fluids from the ipsilateral nostril. It is supposed to be caused by viral infection, but attempts at viral isolation were unsuccessful. Complete spontaneous recovery is usual, taking a few weeks. Our paper seems to be the first report of magnetic resonance imaging of the brain in this condition. It did not disclose any abnormalities in the 2 cases in which it was performed.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/79. Central deafness in a young child with moyamoya disease: paternal linkage in a Caucasian family: two case reports and a review of the literature.A case of 'central deafness' is presented in a 3-year-old male Caucasian child with moyamoya disease (MMD); a rare, progressive and occlusive cerebrovascular disorder predominantly affecting the carotid artery system. documentation of normal peripheral auditory function and brainstem pathway integrity is provided by acoustic admittance, otoacoustic emission and brainstem auditory evoked potential measurements. The lack of behavioral response to sound, and absent middle and long latency auditory evoked potentials suggest thalamo-cortical dysfunction. magnetic resonance imaging showed diffuse ischemic damage in subcortical white matter including areas of the temporal lobes. In addition, there were multiple and focal cortical infarctions in both cerebral hemispheres, focused primarily in the frontal, parietal and temporal areas. Taken together, these structural and functional abnormalities in addition to severely delayed speech and language development are consistent with the diagnosis of central deafness and suggest a disconnection between higher brainstem and cortical auditory areas. The child's father also has MMD, but was diagnosed only recently. The presence of paternal linkage is informative since it rules out x-linked recessive and maternal inheritance. To our knowledge, this represents the first documented case of paternal linkage in MMD with central deafness in a Caucasian child with no apparent Japanese ancestry. Herein, we focus on central auditory dysfunction and consider how lesion-induced changes have contributed to a deficit in basic auditory responsiveness, including a severe disturbance in receptive and expressive auditory-based speech and language skills.- - - - - - - - - - ranking = 3keywords = brain (Clic here for more details about this article) |
3/79. Making requests: illustrations of how right-hemisphere brain damage can affect discourse production.This article examines several factors that influence the production of requests for behavior. Using a role-play methodology, we elicited request productions from well-recovered patients with right-hemisphere brain damage (RHD) and from non brain-damaged control participants. The stimulus items represented variation both on interpersonal factors based on characteristics of the people in the interaction and on situational factors based on what was being requested. A large corpus of responses was elicited from each patient. Responses were coded for request directness, amount of explanatory material over and above the request proper (a relatively demanding method for manipulating the tone of a request), and use of "please" (a relatively simple device for signaling a request). Case-by-case analysis of the patients' performances revealed some common areas of abnormality and also some idiosyncratic features. Some patients produced less explanatory supportive material than control participants, and they tended not to vary the amount of explanatory material as a function of the request scenario. Of interest is that some of the same patients overused "please," and varied their use of this simple device as a function of request scenarios. The discourse strategies observed were likely due to deficits both in pragmatic awareness and in planning utterances. One implication of these results concerns an apt description of the abnormal discourse of RHD patients. The relative lack of supportive explanatory material in their requests may result in patients' seeming rude or inappropriate.- - - - - - - - - - ranking = 6keywords = brain (Clic here for more details about this article) |
4/79. A case of Lafora's disease associated with cardiac arrhythmia.Progressive myoclonic epilepsies are rare, genetically transmitted diseases characterized by epileptic seizures, myoclonus, and progressive neurologic deterioration. Unverricht-Lundborg disease, Lafora's disease, neuronal ceroid lipofuscinosis, mitochondrial disorders, and sialidosis are included in this group. Lafora's disease is a progressive disorder of the central nervous system with onset in the late first or second decade of life and is inherited in an autosomal-recessive pattern. The first clinical manifestation is generalized tonic-clonic seizures, myoclonus, or both, usually seen between the ages of 11 and 18 years. The other clinical manifestations are progressive dementia and limb ataxia. diagnosis is based on showing the typical inclusions in the brain, liver, skin, or muscle tissue specimens. The case of a 6-year-old male patient, who was admitted with the clinical findings of third-degree atrioventricular block and dementia and eventually diagnosed with Lafora's disease, is presented.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
5/79. Foreign Accent syndrome following a catastrophic second injury: MRI correlates, linguistic and voice pattern analyses.A case study of Foreign Accent syndrome (FAS) is presented with discussion of anatomical localization of injury and comparisons of pre- and postinjury linguistic, phonetic, and acoustic speech characteristics. Because the patient's injury and symptoms were unrelated to previously injured left frontal cortex, and in light of another case history (Moonis et al., 1996), we suggest that FAS has a primary subcortical involvement. We also show that this case is accompanied by a deficit in linguistic, but not affective, prosodic expression. We agree that the "foreign" quality of the FAS speech is a perceptual impression of the listener and not inherent in the patient's vocalization. Finally, we suggest a battery of tests for future FAS cases to further our study and understanding of the syndrome.- - - - - - - - - - ranking = 0.69727675936107keywords = injury (Clic here for more details about this article) |
6/79. Auditory neuropathy: case study with hyperbilirubinemia.Auditory neuropathy (AN) has been described in the literature as presenting with a combination of audiometric findings that include elevated behavioral audiometric thresholds, auditory brainstem response findings that are not consistent with audiometric findings, poor speech recognition, and present otoacoustic emissions (OAEs) and/or cochlear microphonics. Since the availability of clinical OAE testing, AN has come to be identified with increasing frequency; however, incidence and prevalence figures are unavailable. There is a great deal of discussion about the accurate diagnosis of AN, its characteristics, and its treatment. Some of this discussion is occurring on the internet and over the telephones. The need to continue to provide information in accessible peer-reviewed journals is paramount. Following a review of the literature, a case study is presented of a boy who was diagnosed with AN as a newborn. He experienced hyperbilirubinemia and other neonatal health complications. His educational intervention was managed elsewhere until recently. Information is presented about the progression of the case over a 5-year period that includes audiologic data and communication development results.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
7/79. Superior written over spoken picture naming in a case of frontotemporal dementia.Two main hypotheses have been proposed regarding the role of phonology in written word production. According to the phonological mediation hypothesis, the retrieval of the lexical phonological representation of a word is an obligatory prerequisite to the retrieval of its spelling. Therefore, deficits to the phonological lexicon should affect both spoken and written picture naming. In contrast, the orthographic autonomy hypothesis posits that the lexical orthographic representations of words can be accessed without any necessary phonological mediation. In support of this view, cases of preserved written naming despite impaired lexical phonology have been reported following brain damage. In this report, we replicate this basic pattern of performance in case YP, a 60-year-old woman with a pattern of frontotemporal dementia. As her disease progressed, YP's ability to write down the names of pictures remained very good despite a severe decline in oral naming. Further testing indicated that this deficit was not primarily due to an articulatory or post-lexical phonological deficit. YP's case provides strong additional support for the orthographic autonomy hypothesis. The significance of this case with respect to the characterization of dementia syndromes is discussed.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
8/79. Worster-Drought and congenital perisylvian syndromes-a continuum?A 5-year-old female was evaluated because of severe speech and expressive language delay. On examination, she could hardly speak and communicated through gestures. She manifested severe dysarthria and difficulty in protruding and moving her tongue laterally. She lacked coordination of the swallowing process, with drooling and an increased mental reflex. Her cognitive development was normal, and no associated neurologic dysfunction of the limbs was noted. On follow-up, the child experienced two episodes of seizures at 6 years of age. magnetic resonance imaging of the brain demonstrated perisylvian and frontal polymicrogyria. Clinical and radiologic findings demonstrated a similarity and continuum between congenital suprabulbar paresis (Worster-Drought syndrome) and perisylvian syndrome.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
9/79. Foreign accent-like syndrome during psychotic exacerbations.OBJECTIVE: The goal of this investigation was to describe the neurologic and psychiatric findings in a patient with foreign accent-like syndrome occurring during episodes of psychotic exacerbation. BACKGROUND: Foreign accent syndrome has been reported in several patients with disorders such as cerebral infarction, cerebral hemorrhage, and head trauma but not in a patient whose primary problem was psychosis. Most patients with this syndrome exhibit some degree of aphasia, and some are dysarthric. METHOD: A schizophrenic patient with foreign accent-like syndrome occurring during a psychotic exacerbation was evaluated by examination and interview, language testing, magnetic resonance imaging, electroencephalography, and other investigative methods. RESULTS: The patient exhibited a prominent British accent, which persisted throughout the duration of his psychotic exacerbation and resolved with improvement of his psychosis. magnetic resonance imaging revealed no lesions, and no abnormality of language or articulation was present. A single-photon emission computed tomography scan could not be obtained. CONCLUSIONS: This represents the first reported case of a patient with foreign accent-like syndrome during psychotic exacerbations.- - - - - - - - - - ranking = 0.00064314490989503keywords = trauma (Clic here for more details about this article) |
10/79. Subcortical brain mechanisms in speech and language.This paper reviews current research and contemporary theories of subcortical participation in the motor control of speech production and language processing. As a necessary precursor to the discussion of the functional roles of the basal ganglia and thalamus, the neuroanatomy of the basal ganglial-thalamocortical circuitry is described. Contemporary models of hypokinetic and hyperkinetic movement disorders based on recent neuroanatomical descriptions of the multi-segmented circuits that characterise basal ganglion anatomy are described. Reported effects of surgically induced lesions in the globus pallidus and thalamus on speech production are reviewed. In addition, contemporary models proposed to explain the possible contribution of various subcortical structures to language processing are described and discussed in the context of evidence gained from observation of the effects of circumscribed surgically induced lesions in the basal ganglia and thalamus on language function. The potential of studies based on examination of the speech/language outcomes of patients undergoing pallidotomy and thalamotomy to further inform the debate relating to the role of subcortical structures in speech motor control and language processing is highlighted.- - - - - - - - - - ranking = 4keywords = brain (Clic here for more details about this article) |
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