Cases reported "Paresis"

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1/18. Design of a prospective neonatal cohort study of homozygous and double heterozygous factor v Leiden and factor II G20210A.

    BACKGROUND: factor v Leiden (FVL) and Factor II (FII) G20210A represent common risk factors for thromboembolic (TE) events. In children, both venous and arterial TE-events have been associated with the presence of FVL and FII G20210A. In most heterozygous children with TE-events other prothrombotic factors can usually be identified. case reports of children with homozygous FVL, including 3 patients described here, suggest that this genotype may convey a particulary high risk. However, prospective data about the type and frequency of TE-events in such children are lacking. STUDY DESIGN: We have initiated a prospective neonatal cohort study for the homozygous and double heterozygous genotypes for FVL and FII G20210A. The probands and the heterozygous controls are identified by neonatal screening that involves > 98% of the children born in berlin and are followed up in a special out-patient clinic to document details of the clinical history, developmental parameters and the occurrence of TE-events. CONCLUSIONS: This study will provide controlled and unbiased information about the clinical significance of the homozygous and double heterozygous genotypes of these mutations.
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2/18. Alteration of the cortical motor map in a patient with intractable focal seizures.

    patients with epilepsia partialis continua may develop progressive neurological deficits of unclear origin. It is possible that repetitive epileptic spikes induce plastic changes in the cortex analogous to the changes observed following direct microstimulation. A child is reported with focal cortical dysplasia, intractable focal seizures, worsening hemiparesis, and alteration of the cortical motor map over time. At age 7, he underwent cortical motor mapping before partial resection of a seizure focus within the right postcentral gyrus. No deficits were present after surgery, and seizure frequency declined by more than 90%. seizures subsequently worsened and a progressive left hemiparesis developed. Cortical remapping at age 12 showed motor centres for left arm, face, and eye movements in an unusual configuration. The location of the motor representation of the face differed from the location obtained at age 7. This case provides direct electrophysiological evidence of reorganisation of the cortical motor map in the human brain.
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3/18. Reversible hemiparesis associated with the use of topiramate.

    We report a case of a 5-year-old boy with intractable partial seizures who developed a transient hemiparesis, worsening of the electroencephalogram (EEG) and a change in his seizure pattern with increased seizure frequency after receiving topiramate (TPM). Symptoms resolved within a month after TPM was discontinued. Clinicians need to be aware that TPM use may occasionally be associated with focal motor weakness and exacerbation of seizures.
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4/18. Interhemispheric intracranial pressure gradients in massive cerebral infarction.

    We report continuous bilateral intracranial pressure (ICP) monitoring immediately after transtentorial herniation in a patient with massive cerebral infarction to: 1) determine presence and time course of compartmental ICP differences, and 2) to study effects of therapy on both hemispheres. A 55-year-old man admitted with watershed infarctions in the left anterior-middle-posterior cerebral arteries distribution. Initial investigations demonstrated highly narrowed left extracranial internal carotid artery. Eight days later he developed unexplained lethargy and anisocoria. head computerized tomography (CT) showed massive left hemispheric infarction, edema, and midline shift. Bilateral subarachnoid bolts demonstrated equally elevated ICP in both hemispheres. hyperventilation and osmotic therapy produced near-identical ICP reduction bilaterally with resolution of anisocoria. Later, plateau waves and autonomic instability developed. Shortly before loss of brainstem function, interhemispheric ICP gradients (left greater than right) of 30-40 mm Hg developed. intracranial pressure did not equalize prior to brain death determination. Bilateral ICP monitoring did not reveal an interhemispheric ICP gradient soon after transtentorial herniation in massive MCA infarction. The presence of interhemispheric ICP gradients in massive stroke remains unproven and further clinical study is necessary.
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5/18. Nonlesional central lobule seizures: use of awake cortical mapping and subdural grid monitoring for resection of seizure focus.

    OBJECT: Surgical treatment options for intractable seizures caused by a nonlesional epileptogenic focus located in the central sulcus region are limited. The authors describe an alternative surgical approach for treating medically refractory nonlesional perirolandic epilepsy. methods: Five consecutive patients who were treated between 1996 and 2000 for nonlesional partial epilepsy that had originated in the central lobule were studied. The patients' ages ranged from 16 to 56 years (mean 28.6 years; there were four men and one woman). The duration of their epilepsy ranged from 8 to 39 years (mean 20.2 years), with a mean seizure frequency of 19 partial seizures per week. Preoperative assessment included video electroencephalography (EEG) and subtracted ictal-interictal single-photon emission computerized tomography coregistered with magnetic resonance imaging (SISCOM). patients underwent an awake craniotomy stereotactically guided by the ictal EEG and SISCOM studies. Cortical stimulation was used to identify the sensorimotor cortex and to reproduce the patient's aura. A subdural grid was then implanted based on these results. Subsequent postoperative ictal electrocorticographic recordings and cortical stimulation further delineated the site of seizure onset and functional anatomy. During a second awake craniotomy, a limited resection of the epileptogenic central lobule region was performed while function was continuously monitored intraoperatively. One resection was limited to the precentral gyrus, two to the postcentral gyrus, and in two the excisions involved regions of both the pre- and postcentral gyri. In three patients a hemiparesis occurred postsurgery but later resolved. In the four patients whose resection involved the postcentral gyms, transient cortical sensory loss and apraxia occurred, which completely resolved in three. Two patients are completely seizure free, two have experienced occasional nondisabling seizures, and one patient has benefited from a more than 75% reduction in seizure frequency. The follow-up period ranged from 2 to 5.5 years (mean 3.5 years). CONCLUSIONS: A limited resection of the sensorimotor cortex may be performed with acceptable neurological morbidity in patients with medically refractory perirolandic epilepsy. This procedure is an alternative to multiple subpial transections in the surgical management of intractable nonlesional epilepsy originating from the sensorimotor cortex.
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6/18. subacute sclerosing panencephalitis presenting with hemiparesis in childhood: case report.

    subacute sclerosing panencephalitis is a chronic and fatal disease of the central nervous system. Most patients present with progressive psychointellectual disturbances. A 14-month-old girl was admitted to our hospital because of left-sided hemiparesis. During hospitalization, focal and generalized seizures occurred. The electroencephalogram (EEG) revealed that periodic lateralized discharges consisted of polyspike and high-voltage slow waves in the left hemisphere. The clinical and EEG findings and positive serology of measles in the cerebrospinal fluid were consistent with subacute sclerosing panencephalitis. In conclusion, we suggest that subacute sclerosing panencephalitis be considered in the differential diagnosis of focal neurologic signs in infants.
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7/18. Focal polymicrogyria, continuous spike-and-wave discharges during slow-wave sleep and Cohen syndrome: a case report.

    Cohen syndrome is a rare genetic disorder consisting of truncal obesity, hypotonia, mental retardation, characteristic facial appearance and ocular anomalies. Other diagnostic clinical features include narrow hands and feet, low growth parameters, neutropenia and chorioretinal dystrophy. Here, we report an 18-year-old male with Cohen syndrome associated with focal polymicrogyria and continuous spike-and-wave discharges during slow-wave sleep.
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8/18. A variant of multifocal motor neuropathy with acute, generalised presentation and persistent conduction blocks.

    OBJECTIVE: Multifocal motor neuropathy with persistent conduction blocks is classically described as a chronic neuropathy with progressive onset, and acute forms have not previously been characterised. We report four cases of severe motor impairment with acute and generalised onset and with persistent motor conduction blocks. patients AND RESULTS: An acute tetraparesis with diffuse areflexia but little or no sensory disturbance was the clinical picture. Serial electrophysiological tests showed persistent multifocal motor conduction blocks with absent F waves in most tested motor nerves. No or minor abnormalities of the sensory nerve action potentials were observed. cerebrospinal fluid contained normal or mildly increased protein levels (<1 g/l) without cells. campylobacter jejuni serology was negative in three patients and consistent with past infection in one patient. Anti-ganglioside antibodies were positive in three patients. A five day course of intravenous immunoglobulins produced nearly complete symptom resolution in three patients and was ineffective in one patient. CONCLUSION: Because of the persistence of multifocal motor conduction blocks for several weeks or months as the isolated electrophysiological feature, these cases could not be consistent with guillain-barre syndrome or chronic inflammatory demyelinating polyneuropathy. They suggest an original variant of multifocal motor neuropathy with an acute and generalised initial presentation and persistent motor conduction blocks affecting all four limbs.
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9/18. Mapping of spikes, slow waves, and motor tasks in a patient with malformation of cortical development using simultaneous EEG and fMRI.

    We report on the simultaneous and continuous acquisition of EEG and functional MRI data in a patient with a left hemiparesis and focal epilepsy secondary to malformation of cortical development in the right hemisphere. EEG-triggered fMRI localization was previously demonstrated in this patient. In the experiments reported here, 322 spikes maximum at electrode C4 and 126 focal slow waves were identified offline. A hierarchy of models was explored in order to assess the relative contributions of each type of EEG event. Modeling the BOLD response to C4 spikes alone showed an area of activation within the large malformation, adjacent to the area of infolding cortex. However, also modeling slow-waves gave rise to a broader and stronger activation, suggesting that the generators overlap. Motor mapping of the right hand showed activation in the left sensorimotor cortex; left-hand tapping led to a more diffuse area of activation, displaced superiorly into the superior frontal gyrus, and a small area of activation within the lesion. In conclusion, continuous EEG-fMRI is useful to compare the functional mapping of epileptiform activity and eloquent cortices in individual patients.
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10/18. Susacs syndrome.

    We describe a 25-year-old woman that presented with frequent rotational dizziness, visual loss of the right eye one month later, and unilateral deafness one year after. After 2 years, she presented with a right hemiparesis and deafness greater for low frequency tones. magnetic resonance imaging with angiography of the brain showed constriction in the first segment of the cerebral anterior artery diagnosed as Susac's syndrome.
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