Congenital ocular motor apraxia: imaging findings

PURPOSE: To determine the frequency of cerebellar and cerebral abnormalities on brain imaging studies in children with congenital ocular motor apraxia. METHODS: Brain imaging studies were performed in 19 children with typical congenital ocular motor apraxia who were in the care of a visual impairment program at a children's hospital. Independent clinical review categorized the subjects as having partial (n = 10) or expanded (n = 9) congenital ocular motor apraxia on the basis of extent of associated speech or neurodevelopmental problems. Fifteen CT studies and 13 MR examinations of the brain performed in these children were reviewed independently by two pediatric neuroradiologists. Radiologic findings were agreed on by consensus. RESULTS: Cerebellar abnormalities were found in 12 of 19 cases. The cerebellar vermis was small in 10 children. A small cerebellar vermis was the only abnormality in five of 10 children with partial congenital ocular motor apraxia and in two of nine children with expanded congenital ocular motor apraxia. Among seven children with a small vermis examined with high-resolution MR imaging, the inferior portion of the vermis was preferentially involved in each case. Of these seven subjects, none of four with partial congenital ocular motor apraxia but two of three with expanded congenital ocular motor apraxia had an abnormality of the superior portion of the vermis. Miscellaneous supratentorial lesions affecting both gray and white matter were found in six subjects. Five of the 19 children had normal imaging findings. CONCLUSION: Inferior vermian hypoplasia is the most common abnormality in children with congenital ocular motor apraxia.     

Speed of motor execution and apraxia

In Paraguay, the plantar surface of the foot is the most common site for malignant melanoma, as it is in several other populations worldwide, most notably in those of African descent. Here, we report the results of the first case-control study of plantar melanoma, carried out in Paraguay. Sixty incident, histologically confirmed cases of plantar melanoma and 256 hospital controls were recruited in 11 hospitals throughout the country during 1988-93. Information was collected on general demographic, social, and lifestyle variables, on external exposures of feet (shoewear, work activities, injuries), and on some constitutional factors (skin, eye and hair color, and pigmented lesions of the feet). Few of the factors examined appeared to be associated with the risk of plantar melanoma. Adjusted for possible confounders, the strongest association was found for reported injuries (odds ratio [OR] = 40.9, 95 percent confidence interval [CI] = 14.8-112.7) and for occurrence of naevi on the soles (OR = 5.9, CI = 2.5-14.3). Walking barefoot did not seem to contribute to the risk although an outdoor workplace was associated with an increased melanoma occurrence (OR = 2.3, CI = 1.1-4.8). Future studies should be aware of problems of recall bias with respect to previous injuries, and ensure that evaluation of pigmentation of the sole is carried out blind to case/control status.     

Paroxysmal and bizarre motor agitation as manifestation of partial frontal lobe epileptic seizures

Three patients, two men of 21 and 38 years and a woman of 20 years old, showed atypical seizures with motor agitation without tongue bite, incontinence or postictal confusion. After extensive video-EEG registration frontal lobe epilepsia was diagnosed. This is a relatively recently recognized disease entity.     

Diagnostic and therapeutic difficulties in oral apraxia in a 16-year-old girl following severe brain trauma

Diagnostic and therapeutic difficulties are described in a case or oral apraxia in a 16-year-old girl who had numerous skull vault bone with concussion and crushing of cerebral tissue. The authors point out that the process of rehabilitaiton of speech disturbances in oral apraxia is long and complex.     

Clinical diagnosis and management of hormonally responsive oral pregnancy tumor (pyogenic granuloma)

OBJECTIVE: To present a review of our experience and that of the medical literature in the diagnosis and management of oral pregnancy tumor (pyogenic granuloma), the natural History of the disease, its hormonal etiology, histopathologic features and management strategies. STUDY DESIGN: Medical literature review. RESULTS: This common, benign, hyperplastic oral mucosal lesion is much less familiar to gynecologists and obstetricians than to dentists. CONCLUSION: For practitioners engaged in primary care obstetrics and gynecology, routine oral examination and proper identification of pyogenic granuloma are important to avoid misdiagnosis and overtreatment.     

Buccofacial apraxia and left cerebral haemorrhage

We examined 33 patients with left intracerebral haemorrhage to investigate the relations to buccofacial apraxia (BFA). BFA was present in 18 cases at 1 month and disappeared in 3 cases and persisted in 15 cases at 6 month from the onset. The existence of BFA seems to be partially dependent the haematoma volume which may cause the organic damage in lenticulate nucleus, insula, posterior limb or internal capsule and anterior portion of paraventricle white matter. All patients with BFA had aphasia (Broca 6, Wernicke 1, Total 10, Amnestic 1). Aphasia of the patients with transient BFA improved as well as the patients without BFA after BFA disappeared. We suspected that improvement or aphasia might be affected by BFA.     

Motor performance in aphasia and ideomotor apraxia

Motor performance in 11 patients with ideomotor apraxia, 11 with aphasia without such apraxia, and 11 normal controls was compared. These three groups were matched on age, sex, education, severity of aphasia, intelligence, and size of lesion. Measures of aiming, tapping, line-following, and steadiness developed by Schoppe in 1974 were used. Both apraxic and aphasic groups showed difficulties with motor performance, and the data of the apraxic group were poorer than those of the aphasic group. These results were consistent with Liepmann's theory.     

Dynamics of spinal cord motor neuron excitability in epileptic patients following surgical elimination of seizures

The authors present materials of the elaboration of a method of obtaining stable erythrocytic diagnostic agents from semipurified preparations of type-specific antigens of the hemolytic streptococcus of the 1st, 4th, 12th and 18th serotypes. Adequate specificity and high sensitivity of the passive hemagglutination test was shown in examination of the sera of experimental animals. In the sera of healthy children the type-specific antibodies were revealed with a different frequency at various seasons of the year; this was in inverse correlation with the level of scarlet fever morbidity.     

Carbamazepine hypersensitivity and rickettsiosis mimicking Kawasaki disease

We report on two patients whose clinical presentation resembled that of Kawasaki disease. The first patient was a boy with epilepsy, whose symptoms first appeared following treatment with carbamazepine. The second boy had Mediterranean Spotted Fever. The significance of medical history in avoiding overdiagnosis of Kawasaki disease is emphasized.     

Tool use and mechanical problem solving in apraxia

Moorlaas (1928) proposed that apraxic patients can identify objects and can remember the purpose they have been made for but do not know the way in which they must be used to achieve that purpose. Knowledge about the use of objects and tools can have two sources: It can be based on retrieval of instructions of use from semantic memory or on a direct inference of function from structure. The ability to infer function from structure enables subjects to use unfamiliar tools and to detect alternative uses of familiar tools. It is the basis of mechanical problem solving. The purpose of the present study was to analyze retrieval of instruction of use, mechanical problem solving, and actual tool use in patients with apraxia due to circumscribed lesions of the left hemisphere. For assessing mechanical problem solving we developed a test of selection and application of novel tools. Access to instruction of use was tested by pantomime of tool use. Actual tool use was examined for the same familiar tools. Forty two patients with left brain damage (LBD) and aphasia, 22 patients with right brain damage (RBD) and 22 controls were examined. Only LBD patients differed from controls on all tests. RBD patients had difficulties with the use but not with the selection of novel tools. In LBD patients there was a significant correlation between pantomime of tool use and novel tool selection but there were single cases who scored in the defective range on one of these tests and normally on the other. Analysis of LBD patients' lesions suggested that frontal lobe damage does not disturb novel tool selection. Only LBD patients who failed on pantomime of object use and on novel tool selection committed errors in actual use of familiar tools. The finding that mechanical problem solving is invariably defective in apraxic patients who commit errors with familiar tools is in good accord with clinical observations, as the gravity of their errors goes beyond what one would expect as a mere sequel of loss of access to instruction of use.     

Supranuclear gaze palsy and eyelid apraxia in postencephalitic parkinsonism

We describe six patients with clinicopathologically confirmed post-encephalitic parkinsonism (PEP) in whom oculomotor abnormalities developed several years after suffering the initial episode of encephalitis lethargica. Four of the cases had vertical supranuclear gaze palsy and two eyelid apraxia, features typically associated with progressive supranuclear palsy (PSP). Our findings indicate that the presence of gaze palsy alone may not be a reliable clinical discriminator between PEP and PSP. Involvement of the dorsal central gray nucleus, nucleus centralis pontis oralis, nucleus dorsal raphe interpositus, rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), nucleus interstitialis of Cajal, nucleus of the posterior commissure, pedunculopontine nuclei and frontal cortex was observed in several of our PEP cases and may contribute to the oculomotor abnormalities in this disorder. Whether the dorsal tegmental nucleus, caudal to the supratrochlear nucleus, severely affected in all our PEP cases, has a role in vertical gaze needs to be further studied.     

Modality specific naming and gesture disturbances: a case with optic aphasia, bilateral tactile aphasia, optic apraxia and tactile apraxia

As life expectancy increases and members of the postwar generation settle into their fifth decade of life, hormone replacement therapy--estrogen or an estrogen-progestin combination--has become a major research interest. An extensive, but often confusing and even contradictory, literature exists on the uses of hormone replacement for the treatment and prevention of a multitude of difficulties that may be associated with the perimenopausal and postmenopausal periods. These include hot flushes, vaginal changes, urinary tract changes, changes in sexuality, affective or emotional symptoms, changes in the oral mucosa and skin, loss of memory and Alzheimer's disease, bone loss and osteoporosis, and cardiovascular disease. This article reviews the literature in each of these areas. It also reviews studies relating to possible side effects of hormone therapy, including endometrial cancer, gall bladder disease, and breast cancer. The article outlines principles for practitioners to follow in assisting women to make informed and individualized decisions about this therapy. Part II of this article, which will appear in the May/June 1996 issue of the Journal of Nurse-Midwifery, will cover specific therapeutic regimens and their management, as well as alternative therapies and preventive measures.     

Eye tracking disorder in schizophrenia is characterized by specific ocular motor defects and is associated with the deficit syndrome

The objective was to determine the relationships between eye tracking disorder (ETD) in schizophrenia, specific ocular motor measures, and the deficit syndrome. Twenty-five normal comparison subjects and 53 schizophrenic patients had eye movements tested with infrared oculography using a sinusoidal target. Patients were assessed with the Schedule for the Deficit Syndrome. For the patients, the distribution of position root mean square error (a global measure of pursuit) was best fit by a mixture of two normal distributions. This information was used to divide the patients into two subgroups, those with and those without ETD. ETD was almost completely accounted for by several specific ocular motor measures and was significantly associated with the deficit syndrome. The finding that ETD was almost completely accounted for by specific measures bridges a gap of interpretation in this field. ETD and the deficit syndrome of schizophrenia may share a common pathophysiology of cerebral cortical-subcortical circuits.     

Sleep and intra-ictal epileptic electroencephalographic activities

From this review it appears that the slow wave sleep (SWS) increases the mean density of electroencephalographic paroxysmal activities (PA) whatever the epileptic syndrome. This pattern is not marked according to a bell curve among the epileptic population: about half the patients exhibit few or no PA during SWS, 20% show an increase during waking and another 20% during SWS. Begnin epilepsy with centro-temporal spikes is associated with an important sleep PA increase. In partial epilepsy, stage 3 and 4 sleep should increase the PA transmission. In children, a large increase in PA during SWS defines the continuous spike-wave during sleep syndrome, which is also observed in the syndrome of acquired aphasia with epilepsy of Landau-Kleffner; both conditions raise the issue of the neuropsychological consequences of the sleep PA. The sleep effect on the various epileptic models is analysed, showing a mean increase in PA during SWS and during transition between sleep and waking. This evidence is in agreement with the fact that during light sleep thalamocortical loops are functioning with an oscillatory pattern which facilitates PA expression. More hypothetic is the effect of sleep on the discharge rate of epileptic focus and on the cortical diffusion of the epileptic discharges. Gaba certainly participates in the thalamic influence, but its role on PA by the cortical and brain stem inhibition is speculative. Noradrenaline and acetylcholine, implicated in waking, reduce PA activity. Interindividual variations suggest that each epileptic has his own pathological neuronal organisation in which cortex and thalamocortical connexions are variously sensitive to the neurotransmitters implicated in sleep and waking.     

Identification of rat brainstem multisynaptic connections to the oral motor nuclei using pseudorabies virus. III. Lingual muscle motor systems

Oromotor behavior results from the complex interaction between jaw, facial, and lingual muscles. The experiments in this and subsequent papers identify the sources of multisynaptic input to the trigeminal, facial, and hypoglossal motor nuclei. In the current experiments, pseudorabies virus (PRV-Ba) was injected into the jaw-opening (anterior digastric and mylohyoid) and jaw-closing muscles (masseter, medial pterygoid, and temporalis) in bilaterally sympathectomized rats. Injection volumes ranged from 2 to 21 microl with average titers of 2.8 x 10(8) pfu/ml and maximum survival times of 96 h. The labeling patterns and distributions were consistent between each of the individual muscles and muscle groups. A predictable myotopic labeling pattern was produced in the trigeminal motor nucleus (Mo 5). Transneuronally labeled neurons occurred in regions known to project directly to Mo 5 motoneurons including the principal trigeminal sensory and supratrigeminal areas, K?lliker-Fuse region, nucleus subcoeruleus, and the parvicellular reticular formation. Maximum survival times revealed polysynaptic connections from the periaqueductal gray, laterodorsal and pedunculopontine tegmental areas, and the substantia nigra in the midbrain, ventromedial pontine reticular regions including the gigantocellular region and pars alpha and ventralis in the pons and medulla, and the nucleus of the solitary tract, paratrigeminal region, and paramedian field in the medulla. Thus, the results define the structure of the multisynaptic brainstem neural circuits controlling mandibular movement in the rat.