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Carbamazepine-induced tics.

A variety of movement disorders are known to occur in association with carbamazepine (CBZ) therapy in adults and children, but development of tics has been described infrequently and only in patients with underlying Tourette's syndrome or other movement disorders. We report 3 children with epilepsy who developed facial motor tics after initiation of CBZ for complex partial seizures. All 3 had documented CBZ blood levels in the therapeutic range at the time, and none had other symptoms or signs of clinical intoxication. Neurologic examinations were normal in 2 and showed developmental delay of expressive language in the third. Brain imaging was normal in all. After development of the tics in 2, CBZ was continued at the same or higher dose, and the tics abated and then ceased spontaneously < or = 6 months. In the third child, the tics ceased after CBZ discontinuation. These cases demonstrate that CBZ can induce simple motor tics in children. These idiosyncratic reactions may be transient and do not always necessitate drug discontinuation.

Adolescent↗

[Neurological development of very low birth weight infants (<1,501 gr) at two ears of age].

The aim of this study is to analyse different aspects of the psychomotor development, during the first two years of the life, of 56 very low birth weight newborns (less of 1.501 g) that had completed a follow-up program. Of the results we have got, the most important are the following: 4 observations (7%) presented severe sequelae motor disability in a tetraparesis with sensorial defects in 3 of them and behavior disturbance (psychosis) with visual defect in the rest. Three observations (5.3%) presented moderate sequelae: all of them with motor disability (hemi- or tetraparesis) and cognitive deficiency. Me have to point out that in the two groups described above, the pathological signs were detected before the age of 6 months. Nine observations (16%) presented mild sequelae dominating language delayed (7% of whole sample) and transient abnormalities in 7 of them (12.5%). In the subgroup of small for gestational age newborns (21 observations), 28.5% presented mild sequelae, 14% transient abnormalities and no one presented severe or moderate sequelae. Although a long term follow-up is necessary in order to detect learning, cognitive, or behavior disturbances, that can just become evident later, we think that the attention of the deviations of the normality during the two first years of the life, would warn about possible future disturbances.

Child Behavior Disorders↗

Nonalcoholic cirrhosis associated with neuropsychological dysfunction in the absence of overt evidence of hepatic encephalopathy.

Although much is known about the neuropsychological functioning of cirrhotic individuals with Laennec's (alcohol associated) cirrhosis, little is known about the neuropsychological functioning of individuals with nonalcoholic cirrhosis. In the present investigation, we have determined that individuals suffering from chronic nonalcoholic cirrhosis, despite the absence of clinical signs of hepatic encephalopathy, are impaired on neuropsychological tests that measure visuopractic capacity, visual scanning, and perceptual-motor speed. In contrast, intellectual, language, memory, attentional, motor, and learning abilities are intact. In comparison with a chronically ill control group of patients suffering from Crohn's disease, individuals with advanced nonalcoholic cirrhosis exhibit less emotional disturbance, but are more impaired in their daily activities. These findings indicate that individuals with nonalcoholic cirrhosis, even in the absence of overt clinical signs of encephalopathy, manifest neuropsychological impairments and experience significant disruption in the routines of everyday living.

Adult↗

Language processing and memory in ill and well siblings from multiplex families affected with schizophrenia.

The present study was designed to extend the investigation of genetic factors for schizophrenia to cognitive and linguistic signs of central nervous system dysfunction. Of 51 siblings studied from 19 schizophrenia multiplex families, 37 had a DSM-III-R diagnosis of schizophrenia or related schzophrenia spectrum disorder and 14 were well. Controls were 17 unrelated healthy individuals within the same social class and age range. Subjects were tested on measures of memory, attention, reading and expressive language ability. Schizophrenic and spectrum disorder siblings were significantly more impaired in tests of auditory discrimination and memory than their well siblings or controls and displayed significantly reduced syntactic complexity to their speech. While well siblings did not differ from controls on most measures, some aspects of language complexity were reduced. A familial effect was observed for tests of reading ability, attention, some syntactic measures, and short-term memory, although these were not the measures that distinguished patients from controls in this cohort, the scores were not correlated among the ill sibling pairs, and poorer scores did not segregate with schizophrenia within these families. Thus, while some measures of language, memory and attention are deviant in patients with schizophrenia, they may not be heritable and directly related to the genetics of the disorder. Instead, they may be a manifestation of, rather than a vulnerability to, the illness.

Adult↗

[An analysis of the process of language deformation in the beginning stages of schizophrenia].

The author analyzed the process of language deformation in the beginning stages of schizophrenia in three cases. Through this analysis, the author obtained the following results. 1. The first symptoms and signs which appeared at the beginning stages of schizophrenia were the deformation of both perceptions and thoughts. Though schizophrenics could recognize each object separately, deformation made them unable to grasp objects as a coherent structure for perceptions and thoughts. 2. Once the certainty of perceptions and thoughts wavered, the use of language changed. Schizophrenics became fixated on words used by others, and thereby unable to choose language appropriate to the situation. 3. The change of language use made schizophrenics isolated from others. Therefore, schizophrenics tried to communicate with other persons indirectly by using a mere shell of language. And then, they constructed delusions and hallucinations in schizophrenia. Based on these results of the analysis, the author herein investigated the beginning stages of schizophrenia which began in the deformation of perceptions and thoughts by using the Wittgenstein's "Language-game Sprachspiel" hypothesis.

Adolescent↗

Controlled evaluation of rural regional perinatal care: developmental and neurologic outcomes at 1 year.

The effectiveness of a rural regionalized perinatal care (RPC) program was evaluated by a controlled, population-based design. The RPC program, begun in a carefully selected study region in July 1975, evolved into a system of care which included the following major components: identification of high-risk pregnancies and high-risk newborn infants; obstetric and newborn consultation and referral services between Level I, II, and III centers; professional education for physicians, nurses, and other health professionals; and nutrition and social work consultation. Substantial resources were made available from 1975 to 1980 to implement this "total package" of RPC. A matched, control region was identified which, except for the RPC program, was as comparable as possible to the study region. It was hypothesized that the RPC intervention would have the following effects when the study region was compared with the control region: reduction in fetal and neonatal mortality, no increase in postneonatal mortality, and reduction in obstetric and newborn morbidity. These findings were reported previously. This paper presents results of hypothesized reductions in adverse developmental, neurologic, maternal-infant attachment, and selected physical health outcomes. A sample of 447 infants was assessed by an extensive battery of instruments at 1 year, adjusted for gestational age. The most notable findings were mothers' reports of receptive language development and observations of maternal attachment behaviors that significantly favored the study region. No significant differences between study and control regions were observed for Bayley Mental and Motor Scores, abnormal neurologic signs, and the physical health measures.(ABSTRACT TRUNCATED AT 250 WORDS)

Child Behavior↗

Evaluating the effects of bilingual traffic signs on driver performance and safety.

Variable message signs (VMS) can provide immediate and relevant information to road users and bilingual VMS can provide great flexibility in countries where a significant proportion of the population speak an alternative language to the majority. The study reported here evaluates the effect of various bilingual VMS configurations on driver behaviour and safety. The aim of the study was to determine whether or not the visual distraction associated with bilingual VMS signs of different configurations (length, complexity) impacted on driving performance. A driving simulator was used to allow full control over the scenarios, road environment and sign configuration and both longitudinal and lateral driver performance was assessed. Drivers were able to read one- and two-line monolingual signs and two-line bilingual signs without disruption to their driving behaviour. However, drivers significantly reduced their speed in order to read four-line monolingual and four-line bilingual signs, accompanied by an increase in headway to the vehicle in front. This implies that drivers are possibly reading the irrelevant text on the bilingual sign and various methods for reducing this effect are discussed.

Accident Prevention↗

Side of onset of motor symptoms influences cognition in Parkinson's disease.

Studies attempting to relate cognitive impairment to asymmetry of motor symptoms in Parkinson's disease (PD) have found contradictory results. We examined 88 patients with unilateral onset of idiopathic PD who underwent a comprehensive neuropsychological assessment, including language, visuospatial abilities, abstraction and reasoning, attention and mental tracking, set shifting, and memory. Patients whose motor signs began on the left side of the body consistently performed more poorly on the battery of cognitive measures than did patients with right-side onset. Significant differences were found on immediate and delayed verbal recall, word retrieval, semantic verbal fluency, visuospatial analysis, abstract reasoning, attention span, and mental tracking. These differences could not be attributed to differences in the overall severity of motor symptoms at the time of cognitive assessment, or the current pattern of motor asymmetry. This finding suggests that damage to right-hemisphere dopamine systems plays a disproportionately greater role in PD-related cognitive decline than a presumably comparable left-hemisphere dopamine depletion.

Aged↗

Cognitive deficits in beta-thalassemia major.

OBJECTIVES: To assess cognitive functioning in patients affected by beta-thalassemia major (beta-th) by using a neuropsychological battery, and to identify clinical correlates. MATERIAL AND METHODS: Forty-six beta-th patients and 46 controls similar for age, sex, and education participated in the study. All subjects performed a comprehensive neuropsychological battery including tests of abstract reasoning, attention, executive functions, language, constructional/visuospatial skills, and memory. RESULTS: Compared to controls beta-th patients, in particular those showing signs of hemosiderosis, were significantly impaired on all neuropsychological tests. There was no relationship between cognitive performances and signs of deferoxamine toxicity, deferoxamine dosage, and levels of hemoglobin and ferritin, while duration of transfusional therapy and time interval between onset of blood transfusions and onset of chelating treatment correlated with performances of tests assessing abstract reasoning, attention, constructional/visuospatial skills, memory and with the scores of the Mini Mental State Examination. CONCLUSION: Our findings suggest that beta-th is associated with neuropsychological impairment involving multiple cognitive domains and argue for a potential role of hemosiderosis on cognitive functioning.

Adult↗

Neuro-ophthalmology of sphenoid sinus carcinoma.

Three cases of sphenoid sinus carcinoma observed personally and 39 identified in the English language publications are reviewed. Sphenoid carcinoma constitutes only 0.3 per cent of sinus cancer. Its symptoms and signs are nonspecific until the sinus wall is penetrated. Once breached, specific neuro-ophthalmological symptoms and signs ensue, resulting from involvement of anatomically contiguous structures. These are characterized most commonly by the sphenocavernous syndrome and less frequently by isolated sixth nerve palsies and visual loss. Identified best by plain radiography, polytomography and computerized tomography, diagnosis of sphenoid carcinoma requires direct biopsy. Treatment, principally with radiotherapy supplemented by chemotherapy, has been disappointing with most patients dead by three years. The authors believe that in rapidly evolving neuro-ophthalmological syndromes of basal origin, accompanied by intractable headache, sphenoid sinus cancer must be considered.

Blepharoptosis↗

Adrenal carcinosarcoma presenting in a woman with clinical signs of virilization. A case report with immunohistochemical and ultrastructural findings.

An adrenal carcinosarcoma is reported in a 29-year-old female presenting with clinical signs of virilization. This is the first reported case of a functioning adrenal carcinosarcoma in the English language literature. The tumor measured 12.5 cm in greatest dimension, weighed 610 g, and consisted of large areas of typical adrenal cortical carcinoma that was, however, interspersed with multiple foci of sarcoma. Rhabdomyosarcomatous elements were identified and confirmed both immunohistochemically and ultrastructurally. After radical resection, the patient received adjuvant mitotane therapy but developed rapid local and metastatic recurrence. Systemic chemotherapy was unsuccessful, and the patient died 8 months after surgery.

Adrenal Cortex Neoplasms↗

Assessing school readiness through the school-entry screening exam.

Although there exists no perfect screening tool that is predictive of a child's success in school, an office visit prior to school enrollment can detect potential problems. This visit should include a history and physical, and developmental assessment. The developmental assessment involves a sampling of a child's abilities in the areas of language, reasoning, gross and fine motor skills, and personal-social development. Providers must be alert to signs and symptoms indicating impediments to learning. Clinicians who are knowledgeable about trends in education regarding readiness testing, birth-date cutoffs and retention can serve as a valuable resource to parents. The clinician can also be a major contributor to the achievement of national education goals set forth by the Bush administration. Ten tools for assessing children are compared, and their appropriate use is discussed.

Child↗

Synovial chondromatosis in a facet joint of a cervical vertebra.

STUDY DESIGN: A case report of a cervical facet joint synovial chondromatosis. OBJECTIVES: To correlate the radiologic and histologic features of vertebral synovial chondromatosis with review of the literature. SUMMARY OF BACKGROUND DATA: Only two previous cases of vertebral facet joint synovial chondromatosis were found in a review of the English language medical literature. METHODS: A 39-year-old woman had severe cervical pain associated with neurologic signs and symptoms in the left upper extremity. Computed tomographic and magnetic resonance imaging studies were performed. RESULTS: Imaging studies showed lytic defects in the laminae of C3 and C4, with intermediate T1 and high T2 signal intensities. The diagnostic impression was that of a lymphangioma or synovial cyst. A laminectomy showed synovial tissue in both the C3-C4 facet joint and the lamina bone. Histologic examination disclosed synovial chondromatosis. CONCLUSIONS: Synovial chondromatosis of the vertebral spine is quite rare, this being only the third reported example. Direct invasion of the cancellous bone, as in this case, also is a very uncommon feature of chondromatosis. It is emphasized that when radiologic studies demonstrate a lesion with cartilaginous characteristics within or juxtaposed to a joint, synovial chondromatosis, despite its rarity, should be included in the differential diagnosis, regardless of the anatomic site.

Adult↗

Dementia accompanying motor neuron disease.

This study presents a structural and clinical description of a patient with dementia of motor neuron disease (D-MND), and compares and contrasts the neuropsychological characteristics of this patient with those of a group of patients with Alzheimer's disease (AD) matched by severity of dementia. The D-MND patient as well as the AD patients performed abnormally on all tasks that assessed executive/frontal functions. However, the D-MND patient tended to be more impaired than AD patients on tasks that required high speed decisions and on shifting from one strategy to another. Deficits in memory, language, and lexical-semantic abilities were also apparent in this patient. The co-occurrence of signs and symptoms characteristics of 'subcortical' dementia with those of 'cortical' dementia suggest that the executive system dysfunction may be secondary to subcortical pathology.

Alzheimer Disease↗

Corpus callosotomy for epilepsy. II. Neurologic and neuropsychological outcome.

Eighteen uncontrolled epileptic patients had neuropsychological evaluation before and after partial or total corpus callosotomy. In patients with early-onset seizures and signs of severe unilateral CNS dysfunction, callosotomy produced no deficits and several improvements. All patients whose language-dominant hemisphere did not control their dominant hand had impairments in some aspect of speech and language function after callosotomy. In some patients, unilateral deterioration of motor function was observed, and was associated with mild to moderate dysfunction in the contralateral hemisphere (ie, memory impairment or preexisting hemiparesis). Postoperative deficits occurred with partial, as well as total, section.

Adolescent↗

A case of mixed acinar-endocrine carcinoma of the pancreas discovered in an asymptomatic subject.

A 50-yr-old Japanese man was found to have a hypoechoic mass 3 cm in diameter in the pancreatic head on an ultrasonography (US) examination without symptoms. A computed tomography (CT) scan demonstrated a 3-cm solid mass in the pancreatic head, and it was more clearly delineated as a low-density area on enhanced CT. Angiography showed a tumorlike stain, 3 cm in size, in the pancreatic head. The preoperative diagnosis was "special type of pancreatic tumor such as acinar cell carcinoma or non-functioning islet cell tumor." The patient was treated by pylorus-preserving pancreatoduodenectomy. Histological, immunohistochemical, and electron-microscopic studies of the surgical specimen led to a definitive diagnosis of a mixed acinar-endocrine carcinoma. The patient is currently well, with no signs of tumor recurrence, 18 mo after the operation. Our search of the Japanese and English-language literature retrieved only 15 well-documented cases of mixed acinar-endocrine carcinoma. Imaging in the reported cases revealed features of either acinar cell carcinoma or islet cell tumor, or both, which can may be detected even in small tumors more easily than conventional invasive ductal carcinoma of the pancreas because the detectability of this rare tumor on US and CT seems to be good.

Carcinoma↗

[Lewy body dementia: some contributions to the clinical knowledge].

INTRODUCTION: Dementia with Lewy bodies is the second commonest type of dementia. The latest criteria for its diagnosis have recently been established (1996). PATIENTS AND METHODS: We report the clinical findings in 25 patients with probable dementia with Lewy bodies. RESULTS: No sex predominance was seen, onset was after the age of 65 years in 60% of the cases and there was a past history of dementia in 48%. Onset was with cognitive disturbances in 32%, Parkinsonism in 32% and both in 32%. When there was an interval between Parkinsonism and dementia, this was of 3-19 months. The dementia included attention disorder, impaired recall and praxis alterations. Disorders of executive function, behaviour or language, were seen in two thirds of the patients and acalculia in over half. Around 50% showed signs of subcortical involvement different from Parkinsonism. Parkinsonism was basically hypokinetic (96%), with tremor in two thirds of the cases, at rest (29%) postural (12%) or combined (59%), and symmetrical in 41%. Rigidity was only seen in 52% and was slight in most cases. In 16% there was weakness of vertical gaze. In 40% urine or double incontinence occurred, mainly when the dementia was at an intermediate or advanced state. CONCLUSIONS: Dementia with Lewy bodies is a dementia with fronto-temporo-parietal alterations, frequently associated with hypokinetic Parkinsonism (which always shows a lag period with respect to dementia of less than 24 months), hallucinations and a fluctuating course. Other findings supporting this diagnosis were seen in two out of every three patients.

Aged↗