Bone–like Polyethelyne Burr–hole Cover

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.     

Opsoclonus–myoclonus–ataxia syndrome in children

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...     

Age–period–cohort analysis of Swiss suicide data, 1881–2000

At the end of the 19^th 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 19^th century and increase until the first half of the 20^th 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.     

Opsoclonus–myoclonus–ataxia syndrome and HIV seroconversion

Journal of Neurology -     

Characterization of Charcot–Marie–Tooth optic neuropathy

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.     

Balance impairment in pediatric charcot–marie–tooth disease

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     

Glioblastoma arising in a patient with Mayer–Rokitansky–Küster–Hauser syndrome

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.     

17p duplicated Charcot–Marie–Tooth 1A

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.     

Radiological imaging findings of Dyke–Davidoff–Masson syndrome

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.     

A family with IVIg-responsive Charcot–Marie–Tooth disease

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.     

Guillain–Barré syndrome–like–onset neurosarcoidosis positive for immunoglobulin G anti-N-acetylgalactosaminyl-GD1a antibody

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.     

Neural correlates of recovery from Foix–Chavany–Marie syndrome

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.     

Aseptic meningo–radiculo–encephalitis presenting initially with urinary retention

We report three male patients with aseptic meningoencephalo– radiculitis presenting with acute urinary retention.Viral antibody titers for herpes types I and II and the PCR studies were negative. The cerebrospinal fluid revealed elevated myelin basic protein.The serum antibodies against a panel of gangliosides, some of which are known to be associated with acquired demyelinating neuropathies, were all negative.The magnetic resonance imaging (MRI) studies revealed spotty T2 high intensities in the basal ganglia, thalamus and brainstem in two patients. In one patient,meningeal gadolinium enhancement of the conus and cauda equina of the spinal cord was recognized. On urodynamic studies, all patients showed features of atonic bladder with or without detrusor hyperactivity. They were treated conservatively without using steroids or immunoglobulins, and made a remarkable functional recovery with the disappearance of abnormal MRI findings.However, all three were left with erectile dysfunction, and two continued to use self–intermittent catheterization at more than 3–year follow–up.There was no recurrence of symptoms. The underlying causes remain unclear, though they may represent a variant of acute disseminated encephalomyelitis.