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《Neurological research》2013,35(3):333-334
Abstract Objective: Several materials are available for covering burr holes but none of them are ideal with respect to biocompatibility, strength and morbidity. With these properties in mind, our objective was to design a porous polyethylene device, which looked like bone and provides protection and cosmesis while being quick and easy to apply. Methods and Materials/Results: A burr–hole cover was created to cover small cranial defects and craniostomies. Using high–density polyethylene, this cover was designed to resemble the bony structure of the skull. Its porous architecture allows for tissue ingrowth and bony integration. It consists of a cylinder which fits into the burr hole and a cap which can be sutured or anchored with titanium screws. Conclusions: The 'bone–like' burr–hole cover provides adequate protection, biocompatibility and cosmesis and is simple to use. Alternative implants can be toxic to surrounding tissues, costly and time consuming to apply. This high–density polyethylene cover is compatible with surrounding tissue as well as being of a porous nature and the material it is made from offers high tensile strength for adequate protection. 相似文献
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Bhatia Poonam Heim Jennifer Cornejo Patricia Kane Lauren Santiago Jason Kruer Michael C. 《Journal of neurology》2022,269(2):750-757
Journal of Neurology - Opsoclonus–myoclonus–ataxia syndrome is a rare neuroimmunologic disorder typically presenting in previously healthy infants and toddlers. It is characterized by a... 相似文献
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Ajdacic-Gross V Bopp M Gostynski M Lauber C Gutzwiller F Rössler W 《European archives of psychiatry and clinical neuroscience》2006,256(4):207-214
At the end of the 19th century, male suicide rates in Switzerland were as high as the respective rates in recent decades, whereas female suicide
rates were distinctly lower. An age–period–cohort analysis was performed to provide more information about the genderspecific
changes over the last century. Suicide mortality has been reported in Switzerland since 1876 when the standardised registration
of mortality data began. The analysed data cover the period 1881–2000. The statistical analyses were based on log–linear models
and data aggregated by 10–year age–intervals and 10–year periodintervals. The results indicate similar age and period effects
in males and females. The estimates representing age–specific risk increase steadily with age, with intermediate plateaus
in the 20s and the 50s. The period–specific estimates follow the economic cycles. The birth cohort effects are stronger in
males and weaker in females. In the males' estimates, there is a peak in cohorts born around 1840 and a low in cohorts born
some 60–100 years later. The estimates increased again in generations born after World War II. In females, the birth cohort
estimates are low in cohorts born in the first half of the 19th century and increase until the first half of the 20th century. Birth cohort effects remain an intriguing topic in epidemiology of suicide. A better understanding of birth cohort
effects might open new doors to suicide prevention. 相似文献
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Varying degrees of optic neuropathy can be seen in patients with Charcot–Marie–Tooth (CMT) disease. To define and characterize the extent of optic neuropathy in patients with CMT2A and CMT1A, two patients from both sub-classifications were evaluated. All patients underwent complete neuro-ophthalmic examinations, and optical coherence (OCT) measurements of the retinal nerve fiber layer (RNFL) and ganglion cell layer complex (GCC) were obtained, along with pattern visual evoked potential (VEP) and pattern electroretinogram (ERG) recordings. RNFL thickness measurements were decreased in both patients with CMT2A, and normal in both patients with CMT1A. GCC measurements were decreased in both patients with CMT2A, mildly decreased in one patient with CMT1A and normal in the second CMT1A patient. VEP latencies were delayed in one patient with CMT2A and one patient with CMT1A. VEP latencies were immeasurable in the other CMT2A patient and not obtained in the second CMT1A patient. Pattern ERG P50-N95 amplitudes were decreased in both patients with CMT2A and normal in one patient with CMT1A. The pattern ERG was immeasurable in the second patient with CMT1A. The pattern of RNFL and GCC thinning in CMT2A with optic neuropathy, a subset of HMSN VI, closely resembles that seen in other mitochondrial optic neuropathies. 相似文献
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Mayer–Rokitansky–Küster–Hauser syndrome is a rare developmental disorder marked by the congenital absence of the uterus and vagina. The syndrome has been associated with tumors of the female reproductive system, but rarely in other organ systems and to our knowledge, never in the brain. We report a glioblastoma in a 34-year-old patient with Mayer–Rokitansky–Küster–Hauser syndrome. 相似文献
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17p duplicated Charcot–Marie–Tooth 1A 总被引:1,自引:0,他引:1
Marques W Freitas MR Nascimento OJ Oliveira AB Calia L Melo A Lucena R Rocha V Barreira AA 《Journal of neurology》2005,252(8):972-979
The most frequent
type of Charcot–Marie–Tooth
(CMT) neuropathy is that associated
with the 17p11.2–p12 chromosome
duplication, whose characteristics
have been well described in
European and North American
populations. In this study, we analyzed
a Brazilian population exhibiting
the mutation, found in 57
patients from 42 families (79%) of
a cohort of 53 families with demyelinating
CMT. Almost 20% of
the duplicated cases were sporadic.
In 77% of the duplicated families
the mutation event occurred in the
hot spot area of the CMT1A–Rep
region. Forty–five percent of patients
were females, 84% were Caucasians
and 13% of African descent.
Distal limb weakness was the
most frequent abnormality, appearing
in 84% of patients, although
uncommon manifestations
such as severe proximal weakness,
floppy baby syndrome, diaphragmatic
weakness and severe scoliosis
were also observed. One patient
was wheelchair–bound, and three
suffered severe hand weakness.
Sensory abnormalities were detected
in 84% of the cases, but 80%
were unaware of this impairment.
Twelve patients complained of positive
sensory manifestations such
as pain and paresthesias. Progression
was reported by 40%. Motor
conduction velocities in the upper
limbs were always less than 35 m/s,
and less than 30.4 m/s in the peroneal
nerve. The findings of this
study expand the clinical spectrum
of the disease. 相似文献
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Yasuo Miki Masahiko Tomiyama Rie Haga Haruo Nishijima Chieko Suzuki Aiichiro Kurihara Kazuhiro Sugimoto Akihiro Hashiguchi Hiroshi Takashima Masayuki Baba 《Journal of neurology》2013,260(4):1147-1151
We report a family of intravenous immunoglobulin (IVIg)-responsive X-linked Charcot–Marie–Tooth disease Type 1 (CMT1X) with a novel gap junction protein 1 mutation. Two of three siblings in the family complained of subacute motor and sensory impairment, and their symptoms improved after the administration of IVIg. Additional IVIg treatment also resulted in similar improvement. The other also showed a mild improvement on IVIg. It has been suggested that an immune-mediated process is involved in the progression of neuropathy in CMT1X. The finding in our report provides evidence of susceptibility to immune-mediated demyelinating neuropathy in some form of CMT1X. Superimposed demyelinating neuropathy as well as a gradual deterioration of neuropathy over decades can be a clinical manifestation of CMT1X. 相似文献
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Timothy Estilow OTR/L Allan M. Glanzman PT DPT PCS Joshua Burns PhD Ann Harrington PT DPT PhD PCS Kayla Cornett PhD Manoj P. Menezes MD PhD Rosemary Shy MD Isabella Moroni MD Emanuela Pagliano MD Davide Pareyson MD Trupti Bhandari PT Francesco Muntoni MD FRCPCH Matilde Laurá MD PhD Mary M. Reilly MD FRCP Richard S. Finkel MD Kate J. Eichinger DPT PhD David N. Herrmann MBBCH Gregory Troutman MBE Paula Bray PhD Mark Halaki PhD Michael E. Shy MD Sabrina W. Yum MD for the CMTPedS STUDY GROUP 《Muscle & nerve》2019,60(3):242-249
Introduction: Balance impairment contributes to gait dysfunction, falls, and reduced quality of life in adults with Charcot–Marie–Tooth disease (CMT) but has been minimally examined in pediatric CMT. Methods: The CMT Pediatric Scale (CMTPedS) was administered to 520 children with CMT. Associations between balance function (Bruininks–Oseretsky Test of Motor Proficiency [BOT-2]) and sensorimotor and gait impairments were investigated. Results: Daily trips/falls were reported by 42.3% of participants. Balance (BOT-2) varied by CMT subtype, was impaired in 42% of 4-year-olds, and declined with age (P < 0.001). Vibration (P < 0.001), pinprick (P < 0.004), ankle dorsiflexion strength (P < 0.001), and foot alignment (P < 0.004) were associated with BOT-2 balance (adjusted R2 = 0.28). The visual dependence of balance increased with age. Discussion: Balance impairment occurs from a young age in children with CMT. Balance intervention studies are required in pediatric CMT and should consider the degree of sensorimotor impairment, foot malalignment, and visual dependence. Muscle Nerve, 2019 相似文献
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Radiological findings of Dyke–Davidoff–Masson syndrome (DDMS) in patients with different etiologies are presented in our study. The study included 12 patients (seven females, five males) for whom radiological examinations were requested due to reasons such as epilepsy, mental retardation, and/or hemiplegia. CT was performed in 12, MRI in 6, MRA in 1, and DSA in 1 patient. Following imaging findings were evaluated: cerebral and cerebellar involvement (laterality, encephalomalacia), affected territories, ventricular enlargement, sulcal enlargement, calvarial thickening, and paranasal sinus enlargement hyperaeration. Age range of the patients was 5–62 (mean 34.1 ± 21.7). Left hemicrania was affected in eight patients, right hemicrania in four. Ipsilateral calvarial thickening and lateral ventricular dilatation were observed in all patients. 11 patients had ipsilateral frontal sinus hyperaeration, sulcal enlargement and encephalomalacia. Wallerian degeneration of the mesencephalon and middle fossa hypoplasia was seen in ten patients, mastoid hyperaeration, third ventricular enlargement and thalamic involvement in nine, and corpus callosum, basal ganglion injury, and sphenoid sinus hyperaeration in eight. MCA, ACA, and PCA territories were involved in six patients. Only MCA territory involvement was seen in four patients. Cerebellar atrophy was contralateral in two patients. Symmetric bilateral atrophy was observed in one patient. DDMS can be encountered with different radiological findings based on cerebral damage formation process and the extent of damage. Patients may have different levels of cerebral hemiatrophy, ipsilateral carvarial thickening, and lateral ventricular dilatation. 相似文献
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《Journal of clinical neuroscience》2014,21(1):170-172
Anti-ganglioside antibodies have been reported in various peripheral neuropathies, including Guillain–Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, Fisher syndrome, monoclonal gammopathy-associated neuropathy, and other idiopathic neuropathies. To our knowledge, there has been no report of anti-ganglioside-positive sarcoidosis. We report a 62-year-old man with acute weakness of the limbs and sensory disturbance of the right arm and trunk resembling GBS. Soluble interleukin-2 receptor and angiotensin-converting enzyme levels were elevated. Anti-ganglioside antibodies (immunoglobulin G anti-N-acetylgalactosaminyl-GD1a antibody [IgG anti-GalNAc-GD1a antibody]) were detected. Neurophysiological examination demonstrated axonal neuropathy. Bilateral hilar lymphadenopathy was demonstrated on a chest CT scan, and abnormal uptake of 67Gallium was detected by scintigraphy. The ratio of CD4 to CD8 was elevated in bronchoalveolar lavage fluid. Noncaseating epithelioid cell granulomas were detected in a specimen obtained via transbronchial lung biopsy. Because intravenous immunoglobulin did not improve the symptoms, we commenced steroid pulse therapy followed by oral prednisolone therapy. After steroid therapy, he recovered fully. Because the findings in our patient fulfilled the criteria for neurosarcoidosis, we diagnosed his illness as probable neurosarcoidosis. To the best of our knowledge, this is the first patient with GBS–like–onset neurosarcoidosis positive for anti-IgG anti-GalNAc-GD1a antibody. 相似文献
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Tom Theys Sofie Van Cauter Kuan H. Kho Anne-Catherine Vijverman Ronald R. Peeters Stefan Sunaert Johannes van Loon 《Journal of neurology》2013,260(2):415-420
Cerebral reorganization during recovery after stroke has been investigated using functional imaging in patients with subcortical motor stroke. The functional correlates of recovery from anarthria, however, are yet unknown. A 48-year-old male patient recovering from complete anarthria after unilateral right-sided subcortical hemorrhagic stroke is described. The main outcome measures included clinical and neuroimaging data at three different time points (at the onset of symptoms, after 6 weeks and after 6 months). At 6 weeks, increased activations in the right and left frontal operculum were found and were followed by a trend towards normalization of the activation pattern at 6 months. These results suggest a role of anterior opercular regions in recovery from anarthria after subcortical stroke. Moreover, complete recovery is possible after such lesions. 相似文献
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