MAGNETIC RESONANCE IN CHEMISTRY, Issue 10 2010
Marino Vega-Vazquez
Abstract A parallel localized spectroscopy (PALSY) method is presented to speed up the acquisition of multidimensional NMR (nD) spectra. The sample is virtually divided into a discrete number of nonoverlapping slices that relax independently during consecutive scans of the experiment, affording a substantial reduction in the interscan relaxation delay and the total experiment time. PALSY was tested for the acquisition of three experiments 2D COSY, 2D DQF-COSY and 2D TQF-COSY in parallel, affording a time-saving factor of 3,4. Some unique advantages are that the achievable resolution in any dimension is not compromised in any way: it uses conventional NMR data processing, it is not prone to generate spectral artifacts, and once calibrated, it can be used routinely with these and other combinations of NMR spectra. Copyright © 2010 John Wiley & Sons, Ltd. [source]

PARENTS BECOME ACTIVE PARTICIPANTS IN HOME THERAPY PROGRAMS, STRIVING TO MAXIMISE GAINS FOR THEIR CHILDREN WITH CEREBRAL PALSY, GIVEN TIME TO COME TO GRIPS WITH THEIR SITUATION

AUSTRALIAN OCCUPATIONAL THERAPY JOURNAL, Issue 2 2004
Christine Imms
No abstract is available for this article. [source]

Session A: Cerebral Palsy: a Population-Based International Perspective

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 2010
Article first published online: 1 SEP 2010
First page of article [source]

Validity and reliability of the guidelines of the Surveillance of Cerebral Palsy in Europe for the classification of cerebral palsy

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 11 2008
Mary Gainsborough MRCPCH
The validity and reliability of the guidelines of the Surveillance of Cerebral Palsy in Europe (SCPE) for the classification of cerebral palsy (CP) were tested by administering 10 written case vignettes via an interactive web-based link to 30 SCPE partners. There was a moderately good level of agreement (,=0.59) about inclusion as a CP case on the SCPE database. Classification by CP subtype differed in two main areas: assigning spastic versus dyskinetic and judgement of distribution of spastic involvement. Agreement on Gross Motor Function Classification System (GMFCS) level was less good than reported in previous studies. Twenty respondents repeated the test 5 months later and there was good repeatability for case inclusion (,= 0.72) but considerable variation in assignment of CP subtype and GMFCS level. There is a need for further collaborative work and training to improve harmonization of the classification of CP, including examination, application of SCPE guidelines, and register coding. [source]

The Definition and Classification of Cerebral Palsy

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 2007
Article first published online: 23 FEB 200
No abstract is available for this article. [source]

American Academy for Cerebral Palsy & Developmental Medicine 2002

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 8 2002
Article first published online: 13 FEB 200
First page of article [source]

The Gross Motor Function Classification System for Cerebral Palsy: a study of reliability and stability over time

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 5 2000
Ellen Wood MD MSc FRCP(C) Assistant Professor
Children with cerebral palsy (CP) experience a change in motor function with age and development. It is important to consider this expected change in offering a prognosis, or in assessing differences in motor function after an intervention. The Gross Motor Function Classification System for CP (GMFCS) has been developed for these purposes. This study was based on a retrospective chart review of 85 children with CP followed from ,2 to ,12 years of age. The GMFCS was applied to clinical notes by two blinded raters four times throughout the study. Interrater reliability was high (G=0.93). Test-retest reliability was high (G=0.79). The positive predictive value of the GMFCS at 1 to 2 years of age to predict walking by age 12 years was 0.74. The negative predictive value was 0.90. The GMFCS can validly predict motor function for children with CP. The results are discussed in terms of their implications for clinical practice and future research. [source]

Clinical Pearls: Headache and Hypoglossal Nerve Palsy

ACADEMIC EMERGENCY MEDICINE, Issue 6 2004
Steven Dorsey MD
No abstract is available for this article. [source]

Adult-Onset Ophthalmoplegic Migraine with Recurrent Sixth Nerve Palsy: A Case Report

HEADACHE, Issue 10 2006
Marco Mucchiut MD
We describe a patient with ophthalmoplegic migraine and left sixth nerve palsy, in whom disease's onset occurred at middle age. [source]

Treatment Acceptability of Healthcare Services for Children with Cerebral Palsy

JOURNAL OF APPLIED RESEARCH IN INTELLECTUAL DISABILITIES, Issue 5 2007
Norm Dahl
Background, Although treatment acceptability scales in intellectual and developmental disabilities research have been used in large- and small-scale applications, large-scale application has been limited to analogue (i.e. contrived) investigations. This study extended the application of treatment acceptability by assessing a large sample of care givers' perceptions of treatment for children with cerebral palsy (CP) in a real-world setting and tested if responses differed across child characteristics, type of medical service or respondent demographics. Method, One hundred and fifty four care givers' for children with CP rated the acceptability of treatments and related medical services by clinicians working in a multi-disciplinary children's specialty setting using Kazdin's (Journal of Applied Behavior Analysis, 13, 1980, 259) Treatment Evaluation Inventory. Results, There were significant (P < 0.05) differences between male and female respondents' ratings of treatment acceptability. There were no other significant differences for caregiver ratings in relation to child characteristics, type of appointment, severity of CP or other respondent demographic characteristics. Conclusion, Mothers and fathers of children with developmental disabilities may differ in their perceptions of the acceptability of medical treatment services for children with developmental disabilities. Future studies addressing treatment acceptability should expand the scope of demographic information assessed and include items specific to the roles respondents have in providing and coordinating therapeutic regimens for their children's medical needs. [source]

Children and Young People with Cerebral Palsy in Northern Ireland (Birth Years: 1977,97)

JOURNAL OF POLICY AND PRACTICE IN INTELLECTUAL DISABILITIES, Issue 3-4 2005
Efrat Merrick BA
[source]

Midbrain SERT in degenerative parkinsonisms: A 123I-FP-CIT SPECT study,

MOVEMENT DISORDERS, Issue 12 2010
Francesco Roselli MD
Abstract SPECT imaging is widely used for the differential diagnosis of degenerative parkinsonisms by exploiting the high affinitiy of the radiotracer 123I-FP-CIT for the dopamine transporter. Reduced levels of DAT are found in Parkinson Disease (PD), Dementia with Lewy Bodies (DLB), and Progressive Supranuclear Palsy (PSP) compared to in Essential Tremor (ET) and Healthy Controls (HC). However, the extent of the neurodegenerative process may extend beyond nigrostriatal system. We have exploited the affinity of the same radiotracer 123I-FP-CIT for the serotonin transporter to investigate SERT levels in the midbrain of patients with PD, DLB, PSP, and ET compared to HC. Using MRI images as anatomical templates for midbrain uptake quantification, we found a mild decrease in SERT levels in PD compared to ET and HC, with marked inter-individual variability; on the other side, PSP and DLB patients displayed markedly reduced to undetectable levels of SERT, respectively. These findings show that the neurodegenerative process affects serotoninergic neurons in parkinsonisms, with much more severe involvement in DLB than in PD patients, despite the comparable loss of striatal DAT. SERT-dependent 123I-FP-CIT uptake may allow a more comprehensive assessment of neurochemical disturbances in degenerative parkinsonisms and may have a value for differential diagnosis. © 2010 Movement Disorder Society [source]

Unresolved issues relating to the Shaking Palsy on the celebration of James Parkinson's 250th birthday

MOVEMENT DISORDERS, Issue S17 2007
Andrew J. Lees MD
Abstract James Parkinson's Essay on the Shaking Palsy published in 1817 provided the first clear clinical description for the disorder now known throughout the world by his name. His primary reason for publishing his monograph shortly before his retirement from medical practice was to draw the medical profession's attention to a malady, which had not yet been defined as a nosological entity. He also hoped that the eminent anatomists of the day would be stimulated to elucidate the pathological lesion responsible for the clinical picture and that this in turn might lead to a rational cure. The concept of Parkinson's disease remains clinically based and successive generations of neurologists have refined and embellished Parkinson's seminal descriptions. Narrative accounts by affected individuals have also helped physicians understand what it is like to live with Parkinson's disease. For many years, the pathological hallmarks of Parkinson's disease were disputed and there were few clinico-pathological reports with adequate clinical description. However, most neurologists now link severe loss of nigral cells in the ventrolateral tier of the pars compacta of the substantia nigra with bradykinesia and the presence of Lewy bodies in a number of discrete brain stem and cortical regions with Parkinson's disease. There are many unanswered clinical questions relating to Parkinson's disease including the striking heterogeneity and frequent limb asymmetry. It also remains somewhat uncertain whether Parkinson's disease is ever truly unilateral by the time of clinical presentation and whether the hand rather than the foot is the most common site of onset. Hyposmia and visual hallucinations are helpful pointers in distinguishing Parkinson's disease from atypical Parkinsonism and should be specifically enquired about in the history. Simple reliable cultural-specific smell identification batteries are an urgent need and target of clinical research. It remains to be determined whether Alzheimer type dementia as opposed to a dysexecutive syndrome should be considered a part of Parkinson's disease and further detailed clinico-pathological correlative studies are needed. It is also unclear whether autosomal dominant monogenetic Parkinsonism due to synuclein or LRRK-2 mutations will prove to be identical clinically with Parkinson's disease and for the present it is wiser to regard Parkinson's disease as a sporadic disorder. Parkinson was an active political reformer and if alive today would certainly be campaigning to translate more effectively the rich seam of neuroscientific research of the last decade into therapeutic benefits for the rising number of people who are developing the shaking palsy as a result of increasing longevity in the developed world. © 2007 Movement Disorder Society [source]

Feasibility of Gait Event Detection Using Intramuscular Electromyography in the Child with Cerebral Palsy

NEUROMODULATION, Issue 3 2004
Richard T. Lauer PhD
Abstract The objective of this study was to develop and test the feasibility of a model that employs electromyographic (EMG) signals to predict the occurrence of gait events in the child with cerebral palsy (CP). This model could be the basis of a future functional electrical stimulation (FES) control system to assist gait. Two children were implanted with bifilar intramuscular electrodes into the quadriceps muscle bilaterally. Muscle activity and gait parameters were recorded, and a fuzzy inference system was used to correlate EMG to five distinct gait events. For nine of the 10 gait events evaluated, the model predicted gait events to within 82 ms on average, as referenced to the VICON motion analysis system. For eight of the 10 events, prediction errors were 0.3% or less. Results indicate that EMG from the proximal musculature could be used to predict the occurrence of gait events in these two children with CP. [source]

Cerebral Palsy: Results of Surgical Releases Augmented with Electrical Stimulation: A Case Study

NEUROMODULATION, Issue 2 2002
James J. McCarthy MD
Abstract The purpose of this case study was to evaluate a patient with diplegic cerebral palsy who underwent soft tissue lengthening augmented with intramuscular electrical stimulation. This is a prospective case study, pre- and post-test design. The patient underwent soft tissue lengthenings of the lower extremities, augmented with placement of intramuscular neuromuscular electrodes. Baseline, 4-, 8-, and 12-month follow-up data were obtained which included range of motion, manual muscle strength testing, motion analysis, oxygen consumption, Gross Motor Function Measure, and Pediatric Evaluation of Disability Inventory. All measured parameters, except knee extensor strength, improved during the postoperative period (baseline to 4-month follow-up) and continued to improve during the rehabilitative period (4,12 month follow-up), despite no formal therapy or home exercise program during this period. We conclude that surgical releases augmented with electrical stimulation resulted in a satisfactory clinic outcome, and may offer a new approach to the treatment of patients with cerebral palsy. [source]

Using Cochrane reviews for oral diseases

ORAL DISEASES, Issue 7 2010
HV Worthington
Oral Diseases (2010) 16, 592,596 Objectives:, To provide readers with information about the Cochrane Oral Health Group and how the reviews on oral diseases have contributed to guideline developments and the commissioning of trials. Materials and methods:, Examples have been selected from the reviews published on The Cochrane Library. Descriptions are given of how these reviews have been used in guideline development and commissioning of trials. Readers are updated on reviews focused on the management of oral cancer and the new venture of diagnostic test reviews. Results:, Reviews on the management of oral diseases due to cancer treatments have been included in guidelines and changed practice in the UK. Cochrane reviews on Bell's Palsy have led to a randomised controlled trial which has changed the evidence base. The Cochrane review on recall intervals between routine appointments has input into the NICE guideline and resulted in a randomised controlled trial to look at different intervals including a risk-based interval. Conclusion:, We hope this article will give readers information on the work of the Cochrane Oral Health Group and insight into the diversity of reviews in oral diseases. The reviews are successfully being used to change practice and as background for the funding of large-scale clinical trials. [source]

Blepharokymographic Analysis of Eyelid Motion in Bell's Palsy

THE LARYNGOSCOPE, Issue 2 2007
Seung-Ho Choi MD
Abstract Objective: To present characteristics of eyelid motion measured by blepharokymography in Bell's palsy patients and to discuss possible roles and limitations of blepharokymography. Study Design. Retrospective analysis. Methods: The study included 72 patients with Bell's palsy who presented to the Department of Otolaryngology at Asan Medical Center, Seoul, Korea, between April 2002 and March 2005, and who underwent both electroneuronography and blepharokymography. Parameters of eyelid motion were measured using revised blepharokymography. Correlations between blepharokymography and electroneuronography or House-Brackmann grade were examined by Spearman rank correlation and Kendall's ,-b correlation, respectively. Results: Compared with the normal side, all parameters of eyelid motion except opening time were decreased on the palsy side, with peak closing velocity showing the greatest difference (40.2%). On average, paralytic eyelids moved down 6.5 mm in 277 ms with a peak velocity of 55.4 mm/s, whereas normal eyelids moved down 9.7 mm in 214 ms, with a peak velocity of 142.6 mm/s. Subtle paralytic eyelid motion or "lid lag" could be objectively documented by blepharokymography to have longer and gentler downward slopes in displacement curves. Most blepharokymographic parameters correlated with ocular electroneuronography and House-Brackmann grade. Conclusions: Slow or incomplete closure of paralytic eyelids can be graphically and numerically analyzed by blepharokymography. Blepharokymography may be useful for evaluating status, predicting prognosis, and assessing effects of rehabilitative procedures, including gold weight implants in patients with facial palsy. [source]

Modified Tarsorrhaphy for Management of the Eye in Facial Nerve Palsy,

THE LARYNGOSCOPE, Issue 7 2006
Luc G. Morris MD
No abstract is available for this article. [source]

Long-Term Result of the New Endoscopic Vocal Fold Medialization Surgical Technique for Laryngeal Palsy,

THE LARYNGOSCOPE, Issue 2 2006
Koichiro Nishiyama MD
Abstract Objective: The conventional surgical method for a case of unilateral laryngeal nerve paralysis with large glottal gap requires an external cervical incision. In the present study, we developed an endoscopic technique of vocal fold medialization that can make the external incision unnecessary. This procedure of autologous transplantation of fascia into the vocal fold (ATFV) was developed for the successful treatment of unilateral laryngeal nerve paralysis. However, the method seemed to be effective only for patients with a relatively mild glottal gap. Study Design and Methods: In the present study, we modified the method of medialization using the ATFV technique to obtain effective closure of a large glottal gap. To overcome this difficulty, an attempt was made to extend the site of transplantation more posteriorly so as to adduct the vocal process of the arytenoid cartilage in the body of the vocal fold. Results: This new technique was applied to eight cases of patients with unilateral laryngeal paralysis with severe dysphonia. None of the patients showed any evidence of falling off of the graft. Elongation of the maximum phonation time and a decrease in airflow rate during phonation were obtained with improvement in voice quality in all patients 1 year after the surgery. Conclusions: This method, with its less invasive approach, proved to be useful for the treatment of large glottal gap due to unilateral laryngeal nerve paralysis. [source]

Botulinum Toxin, Physical and Occupational Therapy, and Neuromuscular Electrical Stimulation to Treat Spastic Upper Limb of Children With Cerebral Palsy: A Pilot Study

ARTIFICIAL ORGANS, Issue 3 2010
Gerardo Rodríguez-Reyes
Abstract Spasticity has been successfully managed with different treatment modalities or combinations. No information is available on the effectiveness or individual contribution of botulinum toxin type A (BTA) combined with physical and occupational therapy and neuromuscular electrical stimulation to treat spastic upper limb. The purpose of this study was to assess the effects of such treatment and to inform sample-size calculations for a randomized controlled trial. BTA was injected into spastic upper limb muscles of 10 children. They received 10 sessions of physical and occupational therapy followed by 10 sessions of neuromuscular electrical stimulation on the wrist extensors (antagonist muscles). Degree of spasticity using the Modified Ashworth scale, active range of motion, and manual function with the Jebsen hand test, were assessed. Meaningful improvement was observed in hand function posttreatment (P = 0.03). Median spasticity showed a reduction trend and median amplitude of wrist range of motion registered an increase; however, neither of these were significant (P > 0.05). There is evidence of a beneficial effect of the combined treatment. Adequate information has been obtained on main outcome-measurement variability for calculating sample size for a subsequent study to quantify the treatment effect precisely. [source]

Progressive Supranuclear Palsy: Pathology and Genetics

BRAIN PATHOLOGY, Issue 1 2007
Dennis W. Dickson
Progressive supranuclear palsy (PSP) is an atypical Parkinsonian disorder associated with progressive axial rigidity, vertical gaze palsy, dysarthria and dysphagia. Neuropathologically, the subthalamic nucleus and brainstem, especially the midbrain tectum and the superior cerebellar peduncle, show atrophy. The substantia nigra shows loss of pigment corresponding to nigrostriatal dopaminergic degeneration. Microscopic findings include neuronal loss, gliosis and neurofibrillary tangles in basal ganglia, diencephalon and brainstem. Characteristic tau pathology is also found in glia. The major genetic risk factor for sporadic PSP is a common variant in the gene encoding microtubule-associated protein tau (MAPT) and recent studies have suggested that this may result in the altered expression of specific tau protein isoforms. Imaging studies suggest that there may be sensitive and specific means to differentiate PSP from other parkinsonian disorders, but identification of a diagnostic biomarker is still elusive. [source]

Phosphorylated Map Kinase (ERK1, ERK2) Expression is Associated with Early Tau Deposition in Neurones and Glial Cells, but not with Increased Nuclear DNA Vulnerability and Cell Death, in Alzheimer Disease, Pick's Disease, Progressive Supranuclear Palsy and Corticobasal Degeneration

BRAIN PATHOLOGY, Issue 2 2001
I. Ferrer
Abnormal tau phosphorylation and deposition in neurones and glial cells is one of the major features in tau pathies. The present study examines the involvement of the Ras/MEK/ERK pathway of tau phosphorylation in Alzheimer disease (AD), Pick's disease (PiD), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD), by Western blotting, single and double-labelling immunohistochemistry, and p21Ras activation assay. Since this pathway is also activated in several paradigms of cell death and cell survival, activated ERK expression is also analysed with double-labelling immunohistochemistry and in situ end-labelling of nuclear DNA fragmentation to visualise activated ERK in cells with increased nuclear DNA vulnerability. The MEK1 antibody recognises one band of 45 kD that identifies phosphorylation-independent MEK1, whose expression levels are not modified in diseased brains. The ERK antibody recognises one band of 42 kD corresponding to the molecular weight of phosphorylation-independent ERK2; the expression levels, as well as the immunoreactivity of ERK in individual cells, is not changed in AD, PiD, PSP and CBD. The antibody MAPK-P distinguishes two bands of 44 kD and 42 kD that detect phosphorylated ERK1 and ERK2. MAPK-P expression levels, as seen with Western blotting, are markedly increased in AD, PiD, PSP and CBD. Moreover, immunohistochemistry discloses granular precipitates in the cytoplasm of neurones in AD, mainly in a subpopulation of neurones exhibiting early tau deposition, whereas neurones with developed neurofibrillary tangles are less commonly immunostained. MAPK-P also decorates neurones with Pick bodies in PiD, early tau deposition in neurones in PSP and CBD, and cortical achromatic neurones in CBD. In addition, strong MAPK-P immunoreactivity is found in large numbers of tau -positive glial cells in PSP and CBD, as seen with double-labelling immunohistochemistry. Yet no co-localisation of enhanced phosphorylated ERK immunoreactivity and nuclear DNA fragmentation is found in AD, PiD, PSP and CBD. Finally, activated Ras expression levels are increased in AD cases when compared with controls. These results demonstrate increased phosphorylated (active) ERK expression in association with early tau deposition in neurones and glial cells in taupathies, and suggest activated Ras as the upstream activator of the MEK/ERK pathway of tau phosphorylation in AD. [source]

Neurological complications in two children with Lemierre syndrome

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 8 2010
BASHEER PEER MOHAMED
Lemierre syndrome is a distinct clinical syndrome comprising oropharyngeal sepsis and fever, internal jugular vein thrombosis and remote septic metastases caused by Fusobacterium species. The mortality rate was historically high and although use of antibiotics led to a dramatic fall in incidence, a resurgence has been seen recently. A 14-year-old male developed Lemierre syndrome after tonsillitis. There was extensive leptomeningitis, especially over the clivus, causing 6th and 12th cranial nerve palsies, a clinical feature termed the ,clival syndrome'. He also developed an epidural abscess in the cervical spine, which was unsafe for surgical drainage. Conservative treatment with an extended course of antibiotics and anticoagulation for jugular vein thrombosis led to a good recovery. A 15-year-old female developed Lemierre syndrome after a persistent sore throat lasting 7 weeks. She had palsy of the 12th cranial nerve from clival osteomyelitis. She was treated with a 6-week course of antibiotics and anticoagulants leading to almost full recovery at 3-month review. Awareness of the potential neurological complications of Lemierre syndrome and prompt management are crucial in reducing morbidity and mortality in this ,forgotten disease'. [source]

Congenital malformations and the cerebral palsies , déjà vu, but now what?

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 4 2010
EVA ALBERMANArticle first published online: 5 JAN 2010
No abstract is available for this article. [source]

Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 1 2010
MARIA CLARK
Aim, Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, is a permanent movement disorder of the bulbar muscles causing persistent difficulties with swallowing, feeding, speech, and saliva control owing to a non-progressive disturbance in early brain development. As such, it falls within the cerebral palsies. The aim of this study was to describe the physical and neuropsychological profiles of children with WDS. Method, Forty-two children with WDS (26 males, 16 females; mean age 7y 10mo, SD 3y 1mo; range 2y 6mo to 16y 5mo) were studied prospectively using a standard protocol. Results, All of the children had severe bulbar dysfunction; 36 out of 42 had feeding difficulties and 23 of 38 had unintelligible speech, which was poorly compensated for by augmentative communication. There were accompanying disturbances in cognition (mean non-verbal IQ 59), behaviour (12/40 attention-deficit,hyperactivity disorder [ADHD]), social communication (8/42 autism), and epilepsy (12/39). The severity of bulbar dysfunction and impact of additional impairments made it difficult to use formal assessments. Interpretation, WDS causes severe and persistent bulbar dysfunction that is often accompanied by additional impairments, as in other cerebral palsies. Speech prognosis is particularly poor. Early diagnosis with appreciation of the underlying neurology would encourage critical evaluation of interventions and long-term planning to improve outcome. [source]

Lower motor neuron involvement in perisylvian polymicrogyria

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 10 2006
Maria Clark MB BChir MRCP
Congenital bilateral perisylvian polymicrogyria syndrome (CBPS) has a cerebral cortical localization and its phenotype was thought to be purely central. This study of seven children with CBPS (five males, two females; mean age 5y [SD 3y 6mo]; range 1mo-11y 10mo) documents electrophysiological evidence of lower motor neuron involvement in association with congenital contractures (limb or jaw) in six of the seven children studied. This is not an expected association and does not conform to the traditional lesional classification system of the cerebral palsies. Possible pathogenic mechanisms are discussed but this association of upper and lower motor neuron involvement is likely to be a previously unsuspected part of a genetic or other pathogenic sequence. [source]

Ocular complications of neurological therapy

EUROPEAN JOURNAL OF NEUROLOGY, Issue 7 2005
S. Hadjikoutis
Treatments used for several neurological conditions may adversely affect the eye. Vigabatrin-related retinal toxicity leads to a visual field defect. Optic neuropathy may result from ethambutol and isoniazid, and from radiation therapy. Posterior subcapsular cataract is associated with systemic corticosteroids. Transient refractive error changes may follow treatment with acetazolamide or topiramate, and corneal deposits and keratitis with amandatine. Intraocular pressure can be elevated in susceptible individuals by anticholinergic drugs, including oxybutynin, tolterodine, benzhexol, propantheline, atropine and amitriptyline, and also by systemic corticosteroids and by topiramate. Nystagmus, diplopia and extraocular muscle palsies can occur with antiepileptic drugs, particularly phenytoin and carbamazepine. Ocular neuromyotonia can follow parasellar radiation. Congenital ocular malformations can result from in utero exposure to maternally prescribed sodium valproate, phenytoin and carbamazepine. Neurologists must be aware of potential ocular toxicity of these drugs, and appropriately monitor for potential adverse events. [source]

Hereditary neuropathy with liability to pressure palsies associated with central nervous system myelin lesions

EUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2001
J. Dac
Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder most commonly caused by a 1.5-Mb deletion in chromosome 17p11.2 which contains the peripheral myelin protein-22 (PMP22) gene. Mutations resulting in functional loss of one PMP22 gene copy are less frequent. We present a 51-year-old patient with a l.5-Mb deletion in chromosome 17p11.2 who exhibited signs of peripheral as well as central nervous system lesions. He gave a history of recurrent episodes of limb numbness and weakness with spontaneous but incomplete recovery since age 20. His father and two brothers had similar symptoms. Neurological examination revealed signs of multiple mononeuropathy associated with frontal lobe, corticospinal tract and cerebellar dysfunction, as well as signs of initial cognitive impairment. Electrophysiological investigations showed a demyelinating peripheral nerve disease with multiple conduction blocks and conduction disturbances in both optic nerves. Magnetic resonance imaging of the brain revealed multiple subcortical and periventricular foci of myelin lesions. The association of central and peripheral nervous system lesions in this patient indicates a possible role of PMP22 not only in peripheral but also in central nervous system myelin structure. [source]

Lesion of the anterior branch of axillary nerve in a patient with hereditary neuropathy with liability to pressure palsies

EUROPEAN JOURNAL OF NEUROLOGY, Issue 5 2000
S. Simonetti
We report the case of a 30-year-old woman affected by hereditary neuropathy with liability to pressure palsies (HNPP), who developed a painless left axillary neuropathy after sleeping on her left side, on a firm orthopaedic mattress, in her eighth month of pregnancy. Electromyography (EMG) showing neurogenic signs in the left anterior and middle deltoid, and normal findings in the left teres minor, posterior deltoid and other proximal upper limb muscles, demonstrated that the lesion was at the level of the axillary anterior branch. A direct compression of this branch against the surgical neck of the humerus seems the most likely pathogenic mechanism. This is the first documented description of an axillary neuropathy in HNPP. Knowledge of its possible occurrence may be important for prevention purposes. [source]

Bell's palsy during interferon therapy for chronic hepatitis C infection in patients with haemorrhagic disorders

HAEMOPHILIA, Issue 2 2000
Ogundipe
Two adult patients with life-long severe haemorrhagic disorders commenced on interferon-,2b therapy for chronic hepatitis C infection. Both developed Bell's palsy several weeks after commencing therapy, They were started on steroids and, in addition, the first patient discontinued interferon-,2b therapy while the second patient elected to continue with therapy. In both cases facial paralysis improved over the ensuing weeks. Bell's palsy is often idiopathic but has been reported. in association with herpesviruses. It is not a recognised complication of chronic hepatitis B or C infection, or interferon-,2b therapy. However, the interferons are associated with numerous adverse reactions including various neuropsychiatric manifestations and neurological syndromes. There are several reports of nerve palsies, including optic tract neuropathy, occurring during interferon therapy, and immune-based mechanisms are thought to play a role in the aetiopathogenesis. No reports of Bell's palsy in association with interferon therapy were identified in our literature search, although one possible case has been reported to the Committee of Safety in Medicine. Although Bell's palsy in our patients may have occurred by chance, a neuropathic effect of interferon-,2b on the facial nerve cannot be excluded and we urge physicians using interferons to be aware of this potential side-effect. [source]