Monoplegia After Anterior Cervical Discectomy and Fusion - a Case Report
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A Syndrome-Based Clinical Approach for Clerkship Students General Comments 1. This Is Not an All-Inclusive “Cookbook” for Ev
A Syndrome-Based Clinical Approach for Clerkship Students General Comments 1. This is not an all-inclusive “cookbook” for every Neurology patient, but a set of guidelines to help you rationally approach patients with certain syndromes (sets of signs and symptoms which suggest a lesion in particular parts of the nervous system). 2. As you obtain a history and perform a neurological physical exam, try initially to localize all the patient’s signs and symptoms to one, single lesion in the nervous system. It may be surprising that a variety of signs and symptoms, at first glance apparently unrelated, on second thought can localize accurately to a single lesion. If this approach fails, then consider multiple, separate lesions for the patient’s signs and symptoms. 3. The tempo or rate at which signs and symptoms develop or occur often suggests the underlying pathological process. a. sudden onset---favors stroke (ischemia or hemorrhage), seizure, migraine (or other headache syndromes), and trauma b. subacute onset---favors inflammatory, infectious or immune-mediated disorders c. chronic onset---favors degenerative disorders, tumors Toximetabolic disorders, potentially treatable and reversible, may mimic lesions in the nervous system, and can evolve at variable tempos. Hereditary conditions may be congenital (present at birth) and nonprogressive or static, or develop later in life, with variable rates of progression. Family members affected by the same genetic disorder may be remarkably similar with regards to onset and clinical severity, while some genetic disorders vary widely regarding when and how severely family members are affected. 4. In the central nervous system, “positive symptoms or phenomena,” such as flashes of light, or a tingling sensation, suggest “excitation” or increased activity in the nervous system: migraine or seizure. -
Child Neurology: Hereditary Spastic Paraplegia in Children S.T
RESIDENT & FELLOW SECTION Child Neurology: Section Editor Hereditary spastic paraplegia in children Mitchell S.V. Elkind, MD, MS S.T. de Bot, MD Because the medical literature on hereditary spastic clinical feature is progressive lower limb spasticity B.P.C. van de paraplegia (HSP) is dominated by descriptions of secondary to pyramidal tract dysfunction. HSP is Warrenburg, MD, adult case series, there is less emphasis on the genetic classified as pure if neurologic signs are limited to the PhD evaluation in suspected pediatric cases of HSP. The lower limbs (although urinary urgency and mild im- H.P.H. Kremer, differential diagnosis of progressive spastic paraplegia pairment of vibration perception in the distal lower MD, PhD strongly depends on the age at onset, as well as the ac- extremities may occur). In contrast, complicated M.A.A.P. Willemsen, companying clinical features, possible abnormalities on forms of HSP display additional neurologic and MRI abnormalities such as ataxia, more significant periph- MD, PhD MRI, and family history. In order to develop a rational eral neuropathy, mental retardation, or a thin corpus diagnostic strategy for pediatric HSP cases, we per- callosum. HSP may be inherited as an autosomal formed a literature search focusing on presenting signs Address correspondence and dominant, autosomal recessive, or X-linked disease. reprint requests to Dr. S.T. de and symptoms, age at onset, and genotype. We present Over 40 loci and nearly 20 genes have already been Bot, Radboud University a case of a young boy with a REEP1 (SPG31) mutation. Nijmegen Medical Centre, identified.1 Autosomal dominant transmission is ob- Department of Neurology, PO served in 70% to 80% of all cases and typically re- Box 9101, 6500 HB, Nijmegen, CASE REPORT A 4-year-old boy presented with 2 the Netherlands progressive walking difficulties from the time he sults in pure HSP. -
Hereditary Spastic Paraplegia
8 Hereditary Spastic Paraplegia Notes and questions Hereditary Spastic Paraplegia What is Hereditary Spastic Paraplegia? Hereditary Spastic Paraplegia (HSP) is a medical term for a condition that affects muscle function. The terms spastic and paraplegia comes from several words in Greek: • ‘spastic’ means afflicted with spasms (an alteration in muscle tone that results in affected movements) • ‘paraplegia’ meaning an impairment in motor or sensory function of the lower extremities (from the hips down) What are the signs and symptoms of HSP? Muscular spasticity • Individuals with HSP commonly will have lower extremity weakness, spasticity, and muscle stiffness. • This can cause difficulty with walking or a “scissoring” gait. We are grateful to an anonymous donor for making a kind and Other common signs or symptoms include: generous donation to the Neuromuscular and Neurometabolic Centre. • urinary urgency • overactive or over responsive “brisk” reflexes © Hamilton Health Sciences, 2019 PD 9983 – 01/2019 Dpc/pted/HereditarySpasticParaplegia-trh.docx dt/January 15, 2019 ____________________________________________________________________________ 2 7 Hereditary Spastic Paraplegia Hereditary Spastic Paraplegia HSP is usually a chronic or life-long disease that affects If you have any questions about DM1, please speak with your people in different ways. doctor, genetic counsellor, or nurse at the Neuromuscular and Neurometabolic Centre. HSP can be classified as either “Uncomplicated HSP” or “Complicated HSP”. Notes and questions Types of Hereditary Spastic Paraplegia 1. Uncomplicated HSP: • Individuals often experience difficulty walking as the first symptom. • Onset of symptoms can begin at any age, from early childhood through late adulthood. • Symptoms may be non-progressive, or they may worsen slowly over many years. -
Functional Neurologic Disorders and Related Disorders Victor W Mark MD ( Dr
Functional neurologic disorders and related disorders Victor W Mark MD ( Dr. Mark of the University of Alabama at Birmingham has no relevant financial relationships to disclose. ) Originally released April 18, 2001; last updated December 13, 2018; expires December 13, 2021 Introduction This article includes discussion of psychogenic neurologic disorders, functional neurologic disorder, functional movement disorder, conversion disorder, and hysteria. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations. Overview Several behavioral disorders are related by (1) their resemblance to other, more familiar neurologic disorders; (2) lack of well-established biomarkers (eg, structural lesions on brain imaging studies, seizure waveforms on EEGs); and (3) aggravation of symptoms with the patient s attention to the disorder. However, the features and causes for these disorders are very different among themselves. This topic reviews functional neurologic disorder, Munchausen syndrome, Munchausen syndrome by proxy, and Ganser syndrome. Key points • Functional neurologic disorders are commonly encountered in general neurologic practices and, hence, knowing their manifestations and treatment is crucial for clinical care. • The disturbance is involuntary, yet at the same time it can be controlled by the patient intermittently. • Despite being self-controllable, the disturbance is generally disabling unless expert professional care is provided. • There is no consistent association between functional neurologic disorder and either posttraumatic emotional stress or sexual abuse. • Functional neurologic disturbances disorder responds best to empathetic concern by the clinician; demonstration that the disorder lacks a structural or permanent etiology; explanation that it can be improved with distraction; and guided attempts to reduce triggers of onset. Cognitive behavioral therapy, combined with physical therapy when warranted, is emerging as a successful intervention. -
Approach to a Patient with Hemiplegia and Monoplegia
CHAPTER Approach to a Patient with Hemiplegia and Monoplegia 27 Sudhir Kumar, Subhash Kaul INTRODUCTION 4. Injury to multiple cervical nerve roots. Monoplegia and hemiplegia are common neurological 5. Functional or psychogenic. symptoms in patients presenting to the emergency department as well as outpatient department. Insidious onset, gradually progressive monoplegia affecting lower limb can be caused by the following Monoplegia refers to weakness of one limb (either arm or conditions: leg) and hemiplegia refers to weakness of one arm and leg on the same side of body (either left or right side). 1. Tumor of the contralateral frontal lobe. There are a variety of underlying causes for monoplegia 2. Tumor of spinal cord at thoracic or lumbar level. and hemiplegia. The causes differ in different age groups. 3. Chronic infection of brain (frontal lobe) or spinal The causes also differ depending on the onset, progression cord (thoracic or lumbar level), such as tuberculous. and duration of weakness. Therefore, one needs to adopt a systematic approach during history taking and 4. Lumbosacral-plexopathy, due to diabetes mellitus. examination in order to arrive at the correct diagnosis. Insidious onset, gradually progressive monoplegia, Appropriate investigations after these would confirm the affecting upper limb, can be caused by one of the following diagnosis. conditions: The aim of this chapter is to systematically look at the 1. Tumor of the contralateral parietal lobe. differential diagnosis of monoplegia and hemiplegia and outline the approach needed to pinpoint the exact 2. Compressive lesion (tumor, large disc, etc) in underlying cause. cervical cord region. 3. Chronic infection of the brain (parietal lobe) or APPROACH TO THE DIAGNOSIS OF MONOPLEGIA spinal cord (cervical region), such as tuberculous. -
KIF1A Missense Mutations in SPG30, an Autosomal Recessive Spastic Paraplegia: Distinct Phenotypes According to the Nature of the Mutations
European Journal of Human Genetics (2012) 20, 645–649 & 2012 Macmillan Publishers Limited All rights reserved 1018-4813/12 www.nature.com/ejhg ARTICLE KIF1A missense mutations in SPG30, an autosomal recessive spastic paraplegia: distinct phenotypes according to the nature of the mutations Stephan Klebe1,2,3,4,5,6, Alexander Lossos7, Hamid Azzedine1,3,4, Emeline Mundwiller1,3,4, Ruth Sheffer7, Marion Gaussen1,3,4, Cecilia Marelli2, Magdalena Nawara1,3,4, Wassila Carpentier8, Vincent Meyer9,10, Agne`s Rastetter1,3,4,11, Elodie Martin1,3,4,11, Delphine Bouteiller1,3,4, Laurent Orlando1,3,4,11, Gabor Gyapay9, Khalid H El-Hachimi1,3,4,11, Batel Zimmerman7, Moriya Gamliel7, Adel Misk12, Israela Lerer7, Alexis Brice*,1,2,3,4,6, Alexandra Durr1,2,3,4,6 and Giovanni Stevanin*,1,2,3,4,11 The hereditary spastic paraplegias (HSPs) are a clinically and genetically heterogeneous group of neurodegenerative diseases characterised by progressive spasticity in the lower limbs. The nosology of autosomal recessive forms is complex as most mapped loci have been identified in only one or a few families and account for only a small percentage of patients. We used next-generation sequencing focused on the SPG30 chromosomal region on chromosome 2q37.3 in two patients from the original linked family. In addition, wide genome scan and candidate gene analysis were performed in a second family of Palestinian origin. We identified a single homozygous mutation, p.R350G, that was found to cosegregate with the disease in the SPG30 kindred and was absent in 970 control chromosomes while affecting a strongly conserved amino acid at the end of the motor domain of KIF1A. -
Myelopathy—Paresis and Paralysis in Cats
Myelopathy—Paresis and Paralysis in Cats (Disorder of the Spinal Cord Leading to Weakness and Paralysis in Cats) Basics OVERVIEW • “Myelopathy”—any disorder or disease affecting the spinal cord; a myelopathy can cause weakness or partial paralysis (known as “paresis”) or complete loss of voluntary movements (known as “paralysis”) • Paresis or paralysis may affect all four limbs (known as “tetraparesis” or “tetraplegia,” respectively), may affect only the rear legs (known as “paraparesis” or “paraplegia,” respectively), the front and rear leg on the same side (known as “hemiparesis” or “hemiplegia,” respectively) or only one limb (known as “monoparesis” or “monoplegia,” respectively) • Paresis and paralysis also can be caused by disorders of the nerves and/or muscles to the legs (known as “peripheral neuromuscular disorders”) • The spine is composed of multiple bones with disks (intervertebral disks) located in between adjacent bones (vertebrae); the disks act as shock absorbers and allow movement of the spine; the vertebrae are named according to their location—cervical vertebrae are located in the neck and are numbered as cervical vertebrae one through seven or C1–C7; thoracic vertebrae are located from the area of the shoulders to the end of the ribs and are numbered as thoracic vertebrae one through thirteen or T1–T13; lumbar vertebrae start at the end of the ribs and continue to the pelvis and are numbered as lumbar vertebrae one through seven or L1–L7; the remaining vertebrae are the sacral and coccygeal (tail) vertebrae • The brain -
Absence of Neurobehavioral Disturbance in a Focal Lesion of the Left Paracentral Lobule
Behavioural Neurology (1992), 5,189-191 ICASE REPORTI Absence of neurobehavioral disturbance in a focal lesion of the left paracentral lobule T. Imamura and K. Tsuburaya Department of Neurology, Tohoku Kohseinenkin Hospital, Sendai, Japan Correspondence to: T. Imamura, Department of Neurology, Institute of Brain Diseases, Tohoku University School of Medicine, 1-1, Seiryo-machi, Aoba-Ku, Sendai 980, Japan The case of a right-handed woman with an infarcation confined to the left paracentral lobule and sparing the supplementary motor area (SMA) is reported. She presented with a right leg monoplegia and displayed no mutism. The absence of any associ ated neurobehavioral disturbances (mutism, forced grasping, reduced spontaneous arm activity or aphasia raises the possi bility that the left SMA has discrete neurobehavioral functions. Keywords: Medial frontal lobe - Precentral gyrus - Supplementary motor area - Transcortical motor aphasia INTRODUCTION Various kinds of neurobehavioral disturbances associated medial part of the left precentral gyrus, which is adjacent with left medial frontal lesions involving the supplemen to the SMA, to evaluate its possible neurobehavioral tary motor area (SMA) have been reported. Aphasia due to functions. damage of the left medial frontal lobe is characterized by an initial period of mutism followed by a stage of CASE REPORT decreased verbal output and spontaneous initiation with normal articulation (Stuss and Benson, 1986). Forced A 73-year-old right-handed woman with a history of grasping, compulsive manipulation of tools and decreased hypertension and diabetes mellitus suddenly developed a spontaneous limb movements have also been described gait disturbance. On examination, 24 h later, she was alert (Wise, 1984; Feinberg et al., 1992). -
ICD9 & ICD10 Neuromuscular Codes
ICD-9-CM and ICD-10-CM NEUROMUSCULAR DIAGNOSIS CODES ICD-9-CM ICD-10-CM Focal Neuropathy Mononeuropathy G56.00 Carpal tunnel syndrome, unspecified Carpal tunnel syndrome 354.00 G56.00 upper limb Other lesions of median nerve, Other median nerve lesion 354.10 G56.10 unspecified upper limb Lesion of ulnar nerve, unspecified Lesion of ulnar nerve 354.20 G56.20 upper limb Lesion of radial nerve, unspecified Lesion of radial nerve 354.30 G56.30 upper limb Lesion of sciatic nerve, unspecified Sciatic nerve lesion (Piriformis syndrome) 355.00 G57.00 lower limb Meralgia paresthetica, unspecified Meralgia paresthetica 355.10 G57.10 lower limb Lesion of lateral popiteal nerve, Peroneal nerve (lesion of lateral popiteal nerve) 355.30 G57.30 unspecified lower limb Tarsal tunnel syndrome, unspecified Tarsal tunnel syndrome 355.50 G57.50 lower limb Plexus Brachial plexus lesion 353.00 Brachial plexus disorders G54.0 Brachial neuralgia (or radiculitis NOS) 723.40 Radiculopathy, cervical region M54.12 Radiculopathy, cervicothoracic region M54.13 Thoracic outlet syndrome (Thoracic root Thoracic root disorders, not elsewhere 353.00 G54.3 lesions, not elsewhere classified) classified Lumbosacral plexus lesion 353.10 Lumbosacral plexus disorders G54.1 Neuralgic amyotrophy 353.50 Neuralgic amyotrophy G54.5 Root Cervical radiculopathy (Intervertebral disc Cervical disc disorder with myelopathy, 722.71 M50.00 disorder with myelopathy, cervical region) unspecified cervical region Lumbosacral root lesions (Degeneration of Other intervertebral disc degeneration, -
Upper Extremity Function in Persons with Tetraplegia: Relationships
Neurorehabilitation and Research Articles Neural Repair Volume 23 Number 5 June 2009 413-421 © 2009 The Author(s) 10.1177/1545968308331143 Upper Extremity Function in Persons with http://nnr.sagepub.com Tetraplegia: Relationships Between Strength, Capacity, and the Spinal Cord Independence Measure Claudia Rudhe, OT, MSc, and Hubertus J. A. van Hedel, PT, PhD Objective. To quantify the relationship between the Spinal Cord Independence Measure III (SCIM III), arm and hand muscle strength, and hand function tests in persons with tetraplegia. Methods. A total of 29 individuals with tetraplegia (motor level between cervical 4 and thoracic 1; sensory-motor complete and incomplete) participated. The total score, category scores, and separate items of the SCIM III were compared to the upper extremity motor score (UEMS), an extended manual muscle test (MMT) for 11 upper extremity muscles, and 6 functional capacity tests of the hand. Spearman’s correlation coefficients (rs) and regression analyses were performed. Results. The SCIM III sum score correlated well with the sum scores of the 3 tests (rs ≥ .76). The SCIM III self-care category correlated better with the tests (rs ≥ .80) compared to the other categories (rs ≤ .72). The SCIM III self-care item “grooming” highly correlated with muscle strength and hand capacity items (rs ≥ .80). A combination of hand muscle tests and the key grasping task explained over 90% of the variability in the self-care category scores. Conclusions. The SCIM III self-care category reflects upper extremity performance as it contains especially useful and valid items that relate to upper extremity function and capacity tests. -
Hyperpigmentation
J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.46.8.743 on 1 August 1983. Downloaded from Journal of Neurology, Neurosurgery, and Psychiatry 1983;46:743-744 Familial spinocerebellar ataxia with skin hyperpigmentation MICHAEL DARAS, ALAN J TUCHMAN, SHANTHA DAVID From the Department ofNeurology, New York Medical College, Lincoln Hospital, Bronx, New York, USA SUMMARY Previous reports have shown the association between familial spastic paraplegia and hypopigmentation of the skin. A family is reported in which three siblings presented with pro- gressive spastic paraparesis and cerebellar ataxia. All the siblings had large hyperpigmented naevi of the lower extremities while none of the unaffected members had a skin lesion. A definite association appears to exist between heredo-familial ataxias and disordered skin pigmentation, but the exact mechanism remains unclear. Many syndromes with disordered skin pigmentation pin and temperature senses were normal but position and and abnormalities of the nervous system have been vibration sense were decreased in both feet. Routine described. In two recent reports' 2 two families with laboratory investigations, including blood count and familial spastic paraplegia were described, in which chemistry, urinalysis, serum B-12 level, VDRL, EKG, and Protected by copyright. hypopigmentation of the skin was present in the chest, skull and complete spine radiographs were normal. affected members. We describe a family with,late Examination of the spinaflfluid was also normal. Computed onset progressive spastic paraplegia with cerebellar tomography of the head revealed bilateral cerebellar ataxia in which the three affected members have hemispheric atrophy. Motor nerve conduction velocities, hyperpigmented naevi in the lower sensory nerve latencies and electromyogram were normal. -
Cerebellar Disease in the Dog and Cat
CEREBELLAR DISEASE IN THE DOG AND CAT: A LITERATURE REVIEW AND CLINICAL CASE STUDY (1996-1998) b y Diane Dali-An Lu BVetMed A thesis submitted for the degree of Master of Veterinary Medicine (M.V.M.) In the Faculty of Veterinary Medicine University of Glasgow Department of Veterinary Clinical Studies Division of Small Animal Clinical Studies University of Glasgow Veterinary School A p ril 1 9 9 9 © Diane Dali-An Lu 1999 ProQuest Number: 13815577 All rights reserved INFORMATION TO ALL USERS The quality of this reproduction is dependent upon the quality of the copy submitted. In the unlikely event that the author did not send a com plete manuscript and there are missing pages, these will be noted. Also, if material had to be removed, a note will indicate the deletion. uest ProQuest 13815577 Published by ProQuest LLC(2018). Copyright of the Dissertation is held by the Author. All rights reserved. This work is protected against unauthorized copying under Title 17, United States C ode Microform Edition © ProQuest LLC. ProQuest LLC. 789 East Eisenhower Parkway P.O. Box 1346 Ann Arbor, Ml 48106- 1346 GLASGOW UNIVERSITY lib ra ry ll5X C C ^ Summary SUMMARY________________________________ The aim of this thesis is to detail the history, clinical findings, ancillary investigations and, in some cases, pathological findings in 25 cases of cerebellar disease in dogs and cats which were presented to Glasgow University Veterinary School and Hospital during the period October 1996 to June 1998. Clinical findings were usually characteristic, although the signs could range from mild tremor and ataxia to severe generalised ataxia causing frequent falling over and difficulty in locomotion.