The Effects of Reflex Path Length on Clonus Frequency in Spastic Muscles
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Motor Neuron Disease (Amyotrophic Lateral Sclerosis) Arising from Longstanding Primary Lateral Sclerosis
74274ournal ofNeurology, Neurosurgery, and Psychiatry 1995;58:742-744 SHORT REPORT J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.58.6.742 on 1 June 1995. Downloaded from Motor neuron disease (amyotrophic lateral sclerosis) arising from longstanding primary lateral sclerosis R P M Bruyn, J H T M Koelman, D Troost,J M B V de Jong Abstract family history was negative and consanguinity Three men were initially diagnosed as was excluded. Examination disclosed slight having primary lateral sclerosis (PLS), weakness of the left thigh muscles, mild spas- but eventually developed amyotrophic ticity of the legs, knee and ankle cloni, and lateral sclerosis (ALS) after 7-5, 9, and at extensor plantars. Blood chemistry, CSF, and least 27 years. Non-familial ALS and EMG were unremarkable. Magnetic reso- PLS might be different manifestations of nance imaging of the cervical spine was nor- a single disease or constitute completely mal and PLS was diagnosed. Micturition distinct entities. The clinical diagnosis of urgency began in 1987. Examination in 1988 PLS predicts a median survival that is showed definite spastic paraparesis and mild four to five times longer than in ALS. proximal weakness of the legs. Sural and tib- ial nerve somatosensory evoked potentials (J Neurol Neurosurg Psychiatry 1995;58:742-744) (SSEPs) were bilaterally absent and delayed respectively. Visual and brainstem auditory evoked potentials (VEPs, BAEPs), and brain Keywords: amyotrophic lateral sclerosis; primary MRI were normal. By 1990, walking had lateral sclerosis become troublesome and dysarthria was pre- sent; the calves showed fasciculation and Primary lateral sclerosis (PLS), defined as some atrophy. -
The Serotonin Syndrome
The new england journal of medicine review article current concepts The Serotonin Syndrome Edward W. Boyer, M.D., Ph.D., and Michael Shannon, M.D., M.P.H. From the Division of Medical Toxicology, he serotonin syndrome is a potentially life-threatening ad- Department of Emergency Medicine, verse drug reaction that results from therapeutic drug use, intentional self-poi- University of Massachusetts, Worcester t (E.W.B.); and the Program in Medical Tox- soning, or inadvertent interactions between drugs. Three features of the sero- icology, Division of Emergency Medicine, tonin syndrome are critical to an understanding of the disorder. First, the serotonin Children’s Hospital, Boston (E.W.B., M.S.). syndrome is not an idiopathic drug reaction; it is a predictable consequence of excess Address reprint requests to Dr. Boyer at IC Smith Bldg., Children’s Hospital, 300 serotonergic agonism of central nervous system (CNS) receptors and peripheral sero- 1,2 Longwood Ave., Boston, MA 02115, or at tonergic receptors. Second, excess serotonin produces a spectrum of clinical find- [email protected]. edu. ings.3 Third, clinical manifestations of the serotonin syndrome range from barely per- This article (10.1056/NEJMra041867) was ceptible to lethal. The death of an 18-year-old patient named Libby Zion in New York updated on October 21, 2009 at NEJM.org. City more than 20 years ago, which resulted from coadminstration of meperidine and phenelzine, remains the most widely recognized and dramatic example of this prevent- N Engl J Med 2005;352:1112-20. 4 Copyright © 2005 Massachusetts Medical Society. able condition. -
The Clinical Approach to Movement Disorders Wilson F
REVIEWS The clinical approach to movement disorders Wilson F. Abdo, Bart P. C. van de Warrenburg, David J. Burn, Niall P. Quinn and Bastiaan R. Bloem Abstract | Movement disorders are commonly encountered in the clinic. In this Review, aimed at trainees and general neurologists, we provide a practical step-by-step approach to help clinicians in their ‘pattern recognition’ of movement disorders, as part of a process that ultimately leads to the diagnosis. The key to success is establishing the phenomenology of the clinical syndrome, which is determined from the specific combination of the dominant movement disorder, other abnormal movements in patients presenting with a mixed movement disorder, and a set of associated neurological and non-neurological abnormalities. Definition of the clinical syndrome in this manner should, in turn, result in a differential diagnosis. Sometimes, simple pattern recognition will suffice and lead directly to the diagnosis, but often ancillary investigations, guided by the dominant movement disorder, are required. We illustrate this diagnostic process for the most common types of movement disorder, namely, akinetic –rigid syndromes and the various types of hyperkinetic disorders (myoclonus, chorea, tics, dystonia and tremor). Abdo, W. F. et al. Nat. Rev. Neurol. 6, 29–37 (2010); doi:10.1038/nrneurol.2009.196 1 Continuing Medical Education online 85 years. The prevalence of essential tremor—the most common form of tremor—is 4% in people aged over This activity has been planned and implemented in accordance 40 years, increasing to 14% in people over 65 years of with the Essential Areas and policies of the Accreditation Council age.2,3 The prevalence of tics in school-age children and for Continuing Medical Education through the joint sponsorship of 4 MedscapeCME and Nature Publishing Group. -
Spasticity in the Podiatric Patient
CHAPTER II SPASTICITY IN TI{E PODIATRIC PATIENT Lwke D. Cicchinelli, DPM The management of the podiatric patient presenting with diseases at this level such as polio effect the classic lower spasticity can be both vexing and rewarding. Content with a motor neuron syndrome. seemingly good outcome at 1 year postoperarively must be This lower motor neuron condition exhibits in tempered wit-h an appreciation for the potential conse- general that individual muscles may be affected, atrophy quences of a growing limb and the complex interplay is pronounced as the muscles do not receive normal between muscles, tendons and osseous factors. The 10 year innervation, flaccidiry and hypotonia of affected muscles follow up outcome might be very different and the patient with loss of tendon reflexes, plantar or babinski reflex if may have other as yet unrecognized functional aberrations. present is of the normal or flexor type, fasciculations may So how does one treat rhese patients? Many be present representing the sporadic discharge of questions come to mind. Do you transfer a spastic muscle fibers due to diseased and irritable axons and muscle? Is the neuromuscular lesion static or progressive? electromyograms reveal reduced numbers of motor units You are a podiatrist, what about the suprastructural and fibrillations representing isolated activity of individ- deformity such as spastic hip adductors, whar is their affect ual muscle fibers. Sensorium is intact. No clonus is on the lower limb function? Is the foot reducible or rigid? Present. Diseases of lower motor neurons or peripheral \fill a muscle tendon transfer shift the muscle power rhe nerves show distal limb weaknesses such as foot drop or a wrong way causing a resultant different deformiry? Are the steppage gait. -
Neuropathic Pain and Spasticity: Intricate Consequences of Spinal Cord Injury
Spinal Cord (2017) 55, 1046–1050 & 2017 International Spinal Cord Society All rights reserved 1362-4393/17 www.nature.com/sc REVIEW Neuropathic pain and spasticity: intricate consequences of spinal cord injury NB Finnerup Study design: The 2016 International Spinal Cord Society Sir Ludwig Guttmann Lecture. Objectives: The aim of this review is to identify different symptoms and signs of neuropathic pain and spasticity after spinal cord injury (SCI) and to present different methods of assessing them. The objective is to discuss how a careful characterization of different symptoms and signs, and a better translation of preclinical findings may improve our understanding of the complex and entangled mechanisms of neuropathic pain and spasticity. Methods: A MEDLINE search was performed using the following terms: ‘pain’, ‘neuropathic’, ‘spasticity’, ‘spasms’ and ‘spinal cord injury’. Results: This review identified different domains of neuropathic pain and spasticity after SCI and methods to assess them in preclinical and clinical research. Different factors important for pain description include location, onset, pain descriptors and somatosensory function, while muscle tone, spasms, reflexes and clonus are important aspects of spasticity. Similarities and differences between neuropathic pain and spasticity are discussed. Conclusions: Understanding that neuropathic pain and spasticity are multidimensional consequences of SCI, and a careful examination and characterization of the symptoms and signs, are a prerequisite for understanding the relationship -
Myoclonus in Adult Huntington's Disease
8 Sloan MA, Price TR, Randall AM, ec al. Inuacerebral hemor- trate that myoclonus can be a disabling but treatable rhage after a-PA and heparin for acute myocardial infarction: feature in a subset of patients with adult HD. the TIM1 I1 pilot and randomized trial combined experience. Stroke 199O;21:182 Patient Histories 9 Kase CS, ONeal AM, Fisher M, et al. Intracranial hemorrhage T.T. was first evaluated at age 28 years, 2 years after the after use of tissue plasminogen activator for coronary thrombol- onset of cognitive decline and incoordination. The diagnosis ysis. Ann Intern Med 1990;112:17-21 10. Cras P, Kawai M, Siedlak S, et al. Neutonal and microglial of HD was supported by findings of dementia and chorei- involvement in B-amyloid protein deposition in Alzheimer's dis- form movements, and a definite family history of adult onset ease. Am J Pathol 1990;137:241-246 dementia and chorea previously diagnosed as HD (Fig 1). 11. Khachamian ZS. Diagnosis of Alzheimer's disease. Arch Neu- Neuropsychometric testing revealed a full scale IQ (FSIQ) of rol 1985;42:1097-1105 72, and positron emission tomography (PET) demonstrated 12. Mark DB, Hlatky MA, OConnor CM, et al. Administration hypometabolism in the caudate nuclei. Ceruloplasmin, serum of thrombolytic therapy in the community hospital: established and urine copper levels, electroencephalogram (EEG), head principles and unresolved issues. J Am Coll Cardiol 1988;12: computed tomographic (CT) scan, and cerebrospinal fluid 32A-43A (CSF) analysis were normal. Serial examinations over the 13. Vinters HV. Cerebral amyloid angiopathy a critical review. -
Non Epileptic Motor Attacks Mimicking Clonic Seizures in Children
CORE Metadata, citation and similar papers at core.ac.uk Provided by Elsevier - Publisher Connector Seizure 21 (2012) 147–150 Contents lists available at SciVerse ScienceDirect Seizure jou rnal homepage: www.elsevier.com/locate/yseiz Case report Extremely sustained startle-induced clonus: Non epileptic motor attacks mimicking clonic seizures in children with encephalopathy a a a a,b, Francesco Mari , Simone Gana , Francesca Piras , Renzo Guerrini * a Paediatric Neurology Unit and Laboratories, Paediatric Hospital A. Meyer – University of Florence, Florence, Italy b IRCCS Fondazione Stella Maris, Calambrone, Pisa, Italy A R T I C L E I N F O A B S T R A C T Article history: Clonus is a pathological motor pattern characterised by involuntary, rhythmic and brisk muscular Received 29 June 2011 contractions in response to peripheral stimuli producing muscle stretching. It indicates pathological Received in revised form 26 September 2011 involvement of the corticospinal tract and can be considered as a functional spastic movement disorder of Accepted 27 September 2011 variable clinical presentation and duration. We documented severe and prolonged episodes of startle- induced clonic attacks associated with severe apnoea, occurring in three infants with severe Keywords: encephalopathy. The clinical characteristics of such episodes are very similar to those of clonic epileptic Clonus seizures. Video-EEG recordings confirmed the non epileptic origin of the episodes. Previous anti-epileptic Startle-induced seizures drug treatment was unsuccessful but myorelaxing drugs produced a dramatic improvement. Clonic seizures Encephalopathy ß 2011 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved. 1. Introduction distress appeared, requiring intubation and ventilatory assistance. -
ALS - Amyotrophic Lateral Sclerosis
ALS - Amyotrophic Lateral Sclerosis Welcome to Neuroscience Pearls: A publication from the UW Medicine Neurosciences Institute. Our goal is to provide useful information pertinent to your practice. Here we bring you key points related to Amyotrophic Lateral Sclerosis - ALS. Richard G. Ellenbogen, MD, FACS, Professor and Chairman, Department of Neurological Surgery, Director, UW Neurosciences Institute Bruce R. Ransom, MD, PhD, Professor and Chair, Department of Neurology, Co-Director, UW Neurosciences Institute, Adjunct Professor, Department of Physiology and Biophysics Contributing Author: B. Jane Distad, MD, Associate Professor, UW Department of Neurology WHAT IS AMYOTROPHIC LATERAL SCLEROSIS? Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive neurodegenerative disorder affecting the motor neuron pathways in the spinal cord, brainstem and brain, leading to weakness and progressive muscle loss. Medical and sup- portive care improves quality of life and life expectancy, but individuals usually succumb to the disease within 5 years. Amyotrophic refers to muscle loss from damage to the motor neurons; lateral indicates the area of the spinal cord affected; and sclerosis means hardening. ALS causes progressive loss of motor neurons, leading to weakness and muscle atrophy in arms, legs - and elsewhere adversely affecting all motor functions including swallowing, speaking and breathing. It is the most common motor neuron disorder. Absolute diagnosis of the disease relies on autopsy features of motor neuron loss and sclerosis. However, the find- ings on physical examination of upper motor and lower motor signs in multiple regions are pathognomonic of ALS (Table 1). These findings in combination with abnormalities in bulbar (the region of the brainstem controlling swallowing and speaking) muscles are highly associated with this particular disease. -
The 2017 ILAE Classification of Seizures Robert S
The 2017 ILAE Classification of Seizures Robert S. Fisher, MD, PhD Maslah Saul MD Professor of Neurology Director, Stanford Epilepsy Center In 2017, the ILAE released a new classification of seizure types, largely based upon the existing classification formulated in 1981. Primary differences include specific listing of certain new focal seizure types that may previously only have been in the generalized category, use of awareness as a surrogate for consciousness, emphasis on classifying focal seizures by the first clinical manifestation (except for altered awareness), a few new generalized seizure types, ability to classify some seizures when onset is unknown, and renaming of certain terms to improve clarity of meaning. The attached PowerPoint slide set may be used without need to request permission for any non-commercial educational purpose meeting the usual "fair use" requirements. Permission from [email protected] is however required to use any of the slides in a publication or for commercial use. When using the slides, please attribute them to Fisher et al. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia doi: 10.1111/epi.13671. ILAE 2017 Classification of Seizure Types Basic Version 1 Focal Onset Generalized Onset Unknown Onset Impaired Aware Motor Motor Awareness Tonic-clonic Tonic-clonic Other motor Other motor Motor Non-Motor (Absence) Non-Motor Non-Motor Unclassified 2 focal to bilateral tonic-clonic 1 Definitions, other seizure types and descriptors are listed in the accompanying paper & glossary of terms 2 Due to inadequate information or inability to place in other categories From Fisher et al. Instruction manual for the ILAE 2017 operational classification of seizure types. -
Muscle Tone Physiology and Abnormalities
toxins Review Muscle Tone Physiology and Abnormalities Jacky Ganguly , Dinkar Kulshreshtha, Mohammed Almotiri and Mandar Jog * London Movement Disorder Centre, London Health Sciences Centre, University of Western Ontario, London, ON N6A5A5, Canada; [email protected] (J.G.); [email protected] (D.K.); [email protected] (M.A.) * Correspondence: [email protected] Abstract: The simple definition of tone as the resistance to passive stretch is physiologically a complex interlaced network encompassing neural circuits in the brain, spinal cord, and muscle spindle. Disorders of muscle tone can arise from dysfunction in these pathways and manifest as hypertonia or hypotonia. The loss of supraspinal control mechanisms gives rise to hypertonia, resulting in spasticity or rigidity. On the other hand, dystonia and paratonia also manifest as abnormalities of muscle tone, but arise more due to the network dysfunction between the basal ganglia and the thalamo-cerebello-cortical connections. In this review, we have discussed the normal homeostatic mechanisms maintaining tone and the pathophysiology of spasticity and rigidity with its anatomical correlates. Thereafter, we have also highlighted the phenomenon of network dysfunction, cortical disinhibition, and neuroplastic alterations giving rise to dystonia and paratonia. Keywords: spasticity; rigidity; dystonia; paratonia 1. Introduction Muscle tone is a complex and dynamic state, resulting from hierarchical and reciprocal anatomical connectivity. It is regulated by its input and output systems and has critical Citation: Ganguly, J.; Kulshreshtha, interplay with power and task performance requirements. Tone is basically a construct of D.; Almotiri, M.; Jog, M. Muscle Tone motor control, upon which power is intrinsically balanced. -
The Five-Minute Neurological Examination
THE FIVE-MINUTE NEUROLOGICAL EXAMINATION Ralph F. Józefowicz, MD Introduction The neurologic examination is considered by many to be daunting. It may seem tedious, time consuming, overly detailed, idiosyncratic, and even capricious. Every neurologist has his/her own version of the examination, and may appear to use “magical thinking” to come up with a diagnosis at the end. In reality, the examination is quite simple. When performing the neurological examination, it is important to keep the purpose of the examination in mind, namely to localize the lesion. A basic knowledge of neuroanatomy is necessary to interpret the examination. The key to performing an efficient neurological examination is observation. More than half of the neurological examination is performed by simply observing the patient – how he/she speaks, thinks, walks, moves, and simply interacts with the examiner. A skillful observer will already localize a lesion, based on simple observations. Formalized testing merely refines the diagnosis, and may only require several additional steps. Performing an overly detailed neurological examination without a purpose in mind is a waste of time, and often yields incidental findings that cloud the picture. The following three pages contain an outline of the components of the five-minute neurological examination, followed by a suggested order for performing this examination. I have also included a detailed handout describing the components of a comprehensive neurological examination, as well as the significance of abnormal findings. Numerous tables are included in this handout to aid in neurological diagnosis. Finally, a series of short cases are included, which illustrate how an efficient and focused neurological examination allows one to make an accurate neurological diagnosis. -
Modified Ashworth Scale and Spasm Frequency Score in Spinal Cord Injury
Spinal Cord (2016) 54, 702–708 & 2016 International Spinal Cord Society All rights reserved 1362-4393/16 www.nature.com/sc ORIGINAL ARTICLE Modified Ashworth scale and spasm frequency score in spinal cord injury: reliability and correlation CB Baunsgaard1,3, UV Nissen1,3, KB Christensen2 and F Biering-Sørensen1 Study design: Intra- and inter-rater reliability study. Objectives: To assess intra- and inter-rater reliability of the Modified Ashworth Scale (MAS) and Spasm Frequency Score (SFS) in lower extremities in a population of spinal cord-injured persons, as well as correlations between the two scales. Setting: Clinic for Spinal Cord Injuries, Rigshospitalet, Hornbaek, Denmark. Methods: Thirty-one persons participated in the study and were tested four times in total with MAS and SFS by three experienced raters. Cohen’s kappa (κ), simple and quadratic weighted (nominal and ordinal scale level of measurement), was used as a measure of reliability and Spearman’s rank correlation coefficient for correlation between MAS and SFS. Results: Neurological level ranged from C2 to L2 and American Spinal Injury Association impairment scale A to D. Time since injury was (mean ± s.d.) 3.4 ± 6.5 years. Age was 48.3 ± 20.2 years. Cause of injury was traumatic in 55% and non-traumatic for 45% of the participants. Antispastic medication was used by 61%. MAS showed intra-rater κsimple = − 0.11 to 0.46 and κweighted = − 0.11 to 0.83. Inter-rater κsimple = − 0.06 to 0.32 and κweighted = 0.08 to 0.74. SFS showed intra-rater κweighted = 0.94 and inter-rater κweighted = 0.93.