Acute Severe Spinal Cord Dysfunction in Bacterial Meningitis in Adults MRI Findings Suggest Extensive Myelitis

Total Page:16

File Type:pdf, Size:1020Kb

Acute Severe Spinal Cord Dysfunction in Bacterial Meningitis in Adults MRI Findings Suggest Extensive Myelitis OBSERVATION Acute Severe Spinal Cord Dysfunction in Bacterial Meningitis in Adults MRI Findings Suggest Extensive Myelitis Stefan Kastenbauer, MD; Frank Winkler, MD; Gunther Fesl, MD; Xaver Schiel, MD; Helmut Ostermann, MD, PhD; Tarek A. Yousry, MD, PhD; Hans Walter Pfister, MD, PhD Background: Bacterial meningitis is rarely compli- the cervical to the lumbar cord. Leptomeningeal and dis- cated by acute spinal cord involvement (eg, myelitis, is- crete nodular intramedullary enhancement on T1- chemic infarction, spinal abscess, or epidural hemor- weighted images was detected only in 1 patient. Fol- rhage). In spinal cord dysfunction, magnetic resonance low-up examinations revealed that hyperintensities imaging (MRI) is the imaging modality of choice. Still, resolved completely in 1 patient, while a central cavita- MRI findings of myelitis due to bacterial meningitis in tion developed in the cervical spinal cord of another, and adults have not been reported. the MRI findings were progressive during the first 4 weeks in the third patient. In all cases, severe paresis and bowel Methods: Spinal MRIs were obtained during the acute and bladder incontinence persisted. stage of meningitis and on follow-up in 3 adults with bac- terial meningitis that was complicated by paraparesis or Conclusion: We demonstrate for the first time the MRI tetraparesis and bowel and bladder incontinence. The findings of adults with acute spinal cord involvement dur- causative pathogens were Streptococcus pneumoniae and ing bacterial meningitis. Magnetic resonance imaging Neisseria meningitidis; in 1 patient, the pathogen was not showed central intramedullary hyperintensities on T2- identified. weighted images that extended from the cervical to the lumbar cord, indicating myelitis. Clinical follow-up ex- Results: In all cases, spinal MRI ruled out a compres- aminations suggest that myelitis during bacterial men- sion of the cord by an extramedullary mass but demon- ingitis has an unfavorable prognosis. strated hyperintensities on T2-weighted images that pre- dominantly involved the gray matter and extended from Arch Neurol. 2001;58:806-810 ACTERIAL MENINGITIS is fre- We describe 3 patients who devel- quently accompanied by oped severe spinal cord dysfunction dur- intracranial complications, ing the acute stage of bacterial meningi- such as cerebrovascular tis and their MRI findings during this involvement, brain edema, condition. Bhydrocephalus, or hearing impairment, as well as systemic complications, such REPORT OF CASES as septic shock, adult respiratory distress syndrome, or disseminated intravascular CASE 1 coagulation.1-3 Spinal cord involvement is a rare complication of bacterial menin- A 36-year-old woman was admitted to a gitis.4 Besides cord compression by a local hospital with a 4-day history of fe- spinal abscess or epidural hemorrhage ver, back pain, and weakness of both legs. following lumbar puncture, the cord can be affected by ischemia due to vascu- For editorial comment litis, shock, herniation, or arachnoiditis, From the Departments of and by myelitis.4 Spinal magnetic reso- see page 717 Neurology (Drs Kastenbauer, nance imaging (MRI) during the acute Winkler, and Pfister), stage of spinal cord dysfunction has been Three years previously, she had under- Neuroradiology (Drs Fesl and reported in only 2 children: no abnor- gone splenectomy for idiopathic throm- Yousry), and Internal Medicine 4 (Drs Schiel and Ostermann), malities were detected in one child, and bocytopenic purpura, and 3 months prior Klinikum Großhadern, enhancement of the cauda equina and to admission, she had received her last Ludwig-Maximilians lumbosacral nerve roots was seen in the pneumococcal vaccination. On admis- University, Munich, Germany. other.5 sion, the patient was febrile, agitated, and (REPRINTED) ARCH NEUROL / VOL 58, MAY 2001 WWW.ARCHNEUROL.COM 806 ©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 confused. Neurologic examination revealed neck stiff- travenous treatment with ceftriaxone was started. His CSF ness, an incomplete bilateral sixth nerve palsy, and a tet- contained 300 cells/µL (71% granulocytes), the protein raparesis (muscle power grade, 4/5 in the arms and 2/5 level was 1.66 g/L, and the CSF glucose level was less than in the legs). Muscle tone and tendon reflexes were de- 40% of the serum glucose level. There were no findings creased in the legs compared with the arms. The plantar on Gram stain and cultures from CSF and blood, but DNA response of the left foot was extensor. Blood cultures were from Neisseria meningitidis was detected in the CSF and positive for Streptococcus pneumoniae. Intravenous ben- blood by polymerase chain reaction. When sedation and zylpenicillin and ceftriaxone were administered, and the ventilator support were discontinued 5 days after admis- patient was transferred to our hospital. The cerebrospi- sion, flaccid paraplegia and a sensory level at T8 were nal fluid (CSF) contained 792 cells/µL (94% granulo- noted. The patient was given intravenous prednisolone cytes), the protein level was 6.3 g/L, and the CSF glu- (150 mg/d for 3 days, then 75 mg/d) for suspected my- cose level was less than 10% of the serum glucose level. elitis. Seventeen days after onset, the patient was trans- Gram-positive diplococci were present in the CSF, and ferred to our department. Despite repeated corticoste- the latex agglutination test result was positive for pneu- roid treatment, the spinal cord dysfunction was mococcal antigen; however, CSF cultures remained ster- progressive until 4 weeks after onset. Seven weeks after ile. Because myelitis was suspected, the patient was given onset, the patient still had a flaccid tetraparesis (muscle intravenous dexamethasone (24 mg/d) for 4 days. The power grade, 4/5 in the arms and 0/5 in the legs) and a clinical course was further complicated by ischemic in- sensory level at T6. Furthermore, bowel and bladder in- farction in the right frontal lobe due to cerebral vascu- continence were present. litis. Three months after the onset of disease, the patient had a spastic tetraparesis (muscle power grade, 4/5 in the METHODS arms and 2/5 in the legs). Sensibility was intact, but blad- der and bowel control were still absent. In all patients, MRI was performed on a 1.5-T MRI scan- ner (Magnetom Vision; Siemens, Munich, Germany). CASE 2 Detailed information on the sequences is given in the figure legends. A 33-year-old man who had been hospitalized because of bronchitis and mild graft-vs-host disease of the liver RESULTS following allogeneic bone marrow transplantation for chronic myeloid leukemia 6 months previously devel- For all 3 patients, T2-weighted images showed intramed- oped fever and headache despite oral antibiotic therapy ullary hyperintensities during the acute stage of spinal (amoxicillin, ciprofloxacin, and fluconazole). Within 24 cord dysfunction (day 5 of meningitis/day 5 of spinal cord hours of admission, weakness of both legs and bowel and dysfunction in patient 1; day 2 of meningitis/day 2 of spi- bladder incontinence appeared. Examination revealed dis- nal cord dysfunction in patient 2; day 6 of meningitis/ crete neck stiffness, spastic paraplegia, and a sensory level after day 1 of spinal cord dysfunction in patient 3), which at C1. The CSF contained 2976 cells/µL (94% granulo- appeared to be most pronounced in the gray matter. The cytes, no malignant cells), the protein level was 1.07 g/L, signal abnormalities extended from the cervical to the lum- and the CSF glucose level was less than 40% of the se- bar cord in patients 1 and 2 and from the cervical to the rum glucose level. There were no findings on Gram stain thoracic cord in patient 3 (Figure 1 and Figure 2). of CSF, and cultures of blood and CSF were sterile. Trans- While swelling of the spinal cord was clearly evident in cranial Doppler sonography and cranial MRI were nor- patient 3, it was not observed in the other patients. In- mal. Nosocomial bacterial meningitis was suspected, and tense leptomeningeal and discrete nodular intramedul- the patient was treated with intravenous vancomycin, me- lary gadolinium enhancement was observed in patient 1 ropenem, metronidazole, and a 4-day regimen of oral but not in patients 2 and 3 (Figure 1A). On native T1- dexamethasone, 24 mg/d. Six weeks after onset of men- weighted images, the central spinal cord lesion ap- ingitis, follow-up examination showed a motor level at peared hypointense in patient 1 and isointense in pa- C8, with a power grade of 4/5 in the small muscles of tients 2 and 3. In none of the patients did we detect an the hand and 2/5 in the legs. No clear sensory level could intramedullary abscess or compression of the cord by a be determined, but sensibility and vibratory sense were mass, such as an epidural hemorrhage, extramedullary decreased in both legs. Bowel and bladder incontinence abscess, or subdural empyema. were still present. On follow-up, the intramedullary signal abnormali- ties had completely resolved in patient 2. In patient 1, the CASE 3 extensive hyperintensities had almost completely re- solved by day 17 after onset of meningitis, but on day 24, A previously healthy 17-year-old boy was admitted to a a central hyperintense lesion was detected in the cervical local hospital with a 1-day history of fever, nausea, and spinal cord at the level of C6. This lesion was interpreted headache. Examination revealed no focal neurologic defi- to be a newly formed syrinx. In patient 3, the T2 signal ab- cit, but a discrete neck stiffness and a petechial rash on normalities that initially involved only the thoracic cord the trunk and extremities. Shortly after admission, he had extended to C4 by day 17 (Figure 2) and to C3 by day 29. a respiratory arrest, at which time no blood pressure could Furthermore, the high intensity of the T2 signal was sub- be detected.
Recommended publications
  • Central Pain in the Face and Head
    P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-128 Olesen- 2057G GRBT050-Olesen-v6.cls August 17, 2005 2:10 ••Chapter 128 ◗ Central Pain in the Face and Head J¨orgen Boivie and Kenneth L. Casey CENTRAL PAIN IN THE FACE AND HEAD Anesthesia dolorosa denotes pain in a region with de- creased sensibility after lesions in the CNS or peripheral International Headache Society (IHS) code and diag- nervous system (PNS). The term deafferentation pain is nosis: used for similar conditions, but it is more commonly used in patients with lesions of spinal nerves. 13.18.1 Central causes of facial pain 13.18.1 Anesthesia dolorosa (+ code to specify cause) 13.18.2 Central poststroke pain EPIDEMIOLOGY 13.18.3 Facial pain attributed to multiple sclerosis 13.18.4 Persistent idiopathic facial pain The prevalence of central pain varies depending on the un- 13.18.5 Burning mouth syndrome derlying disorder (Tables 128-1 and 128-2) (7,29). In the ab- 13.19 Other centrally mediated facial pain (+ code to sence of large scale epidemiologic studies, only estimates specify etiology) of central pain prevalence can be quoted. In the only prospective epidemiologic study of central Note that diagnosis with IHS codes 13.18.1, 13.18.4, and pain, 191 patients with central poststroke pain (CPSP) 13.18.5 may have peripheral causes. were followed for 12 months after stroke onset (1). Sixteen World Health Organization (WHO) code and diagnosis: (8.4%) developed central pain, an unexpectedly high inci- G 44.810 or G44.847.
    [Show full text]
  • Caspr2 Antibodies in Patients with Thymomas
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector MALIGNANCIES OF THE THYMUS Caspr2 Antibodies in Patients with Thymomas Angela Vincent, FRCPath,* and Sarosh R. Irani, MA* neuromuscular junction. Neuromyotonia (NMT) is due to Abstract: Myasthenia gravis is the best known autoimmune disease motor nerve hyperexcitability that leads to muscle fascicula- associated with thymomas, but other conditions can be found in tions and cramps. A proportion of patients have antibodies patients with thymic tumors, including some that affect the central that appear to be directed against brain tissue-derived volt- nervous system (CNS). We have become particularly interested in age-gated potassium channels (VGKCs) that control the ax- patients who have acquired neuromyotonia, the rare Morvan disease, onal membrane potential.4,5 VGKC antibody titers are rela- or limbic encephalitis. Neuromyotonia mainly involves the periph- tively low in NMT. eral nerves, Morvan disease affects both the peripheral nervous Morvan disease is a rare condition first described in system and CNS, and limbic encephalitis is specific to the CNS. 1876 but until recently hardly mentioned outside the French Many of these patients have voltage-gated potassium channel auto- literature.6 The patients exhibit NMT plus autonomic distur- antibodies. All three conditions can be associated with thymomas bance (such as excessive sweating, constipation, and cardiac and may respond to surgical removal of the underlying tumor
    [Show full text]
  • Neurosyphilis Presenting with Myelitis-Case Series and Literature Review
    Neurosyphilis presenting with myelitis-Case series and literature review Yali Wu Capital Medical University Aliated Beijing Ditan Hospital https://orcid.org/0000-0002-9737-6439 Wenqing Wu ( [email protected] ) https://orcid.org/0000-0001-7428-5529 Case report Keywords: Syphilis, Neurosyphilis, Spinal cord, Magnetic resonance imaging Posted Date: April 5th, 2019 DOI: https://doi.org/10.21203/rs.2.1849/v1 License: This work is licensed under a Creative Commons Attribution 4.0 International License. Read Full License Version of Record: A version of this preprint was published at Journal of Infection and Chemotherapy on February 1st, 2020. See the published version at https://doi.org/10.1016/j.jiac.2019.09.007. Page 1/9 Abstract Background Neurosyphilis is a great imitator because of its various clinical symptoms. Syphilitic myelitis is extremely rare manifestation of neurosyphilis and often misdiagnosed. However, a small amount of literature in the past described its clinical manifestations and imaging features, and there was no relevant data on the prognosis, especially the long-term prognosis. In this paper, 4 syphilis myelitis patients admitted to our hospital between July 2012 and July 2017 were retrospectively reviewed. In the 4 patients, 2 were females, and 2 were males. We present our experiences with syphilitic myelitis, discuss the characteristics, treatment and prognosis. Case presentation The diagnosis criteria were applied: (1) diagnosis of myelitis established by two experienced neurologist based on symptoms and longitudinally extensive transverse myelitis (LETM) at the cervical and thoracic levels mimicked neuromyelitis optic (NMO) on magnetic resonance imaging (MRI) ; (2) Neurosyphilis (NS) was diagnosed by positive treponema pallidum particle assay (TPPA) and toluidine red untreated serum test (TRUST) in the serum and CSF; (3) negative human immunodeciency virus (HIV).
    [Show full text]
  • 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,
    [Show full text]
  • Transverse Myelitis Interagency Collaboration
    SHNIC Specialized Health Needs Factsheet: Transverse Myelitis Interagency Collaboration What is it? Transverse Myelitis is a neurological disorder caused by inflammation of the spinal cord. A child will experience weakness, pain, sensory and autonomic dysfunction. Auto- nomic, involuntary activities such as breathing, digestion, heartbeat and reflexes can be affected. Symptoms can ap- pear suddenly within hours or progress over a span of sev- eral weeks. Approximately 25% of cases are children. A peak in incidence occurs between ages of 10 and 19 and females are at higher risk than men. During an inflammato- ry response the myelin, or protective fatty coating on nerve cells, is damaged or destroyed. TM can also be the first symptom to diagnose Multiple Sclerosis. What causes it? Researchers believe it is the body’s immune response, not the infection itself, that causes the inflammatory response. This indicates an auto-immune reaction, where the body attacks it’s own tissue rather than the infection, is responsible. Research has made connections to the damage of spinal nerves following a viral or bacterial infection, especially those associated with a rash. According to the National Institute of Neurologic Disorders and Stroke, infectious agents sus- pected of causing TM include Varicella zoster (the virus that causes chickenpox and shingles), Herpes simplex, Cytomegalovirus, Epstein-Barr, Influenza, Echovirus, Human immunodefi- ciency virus (HIV), Hepatitis A, and Rubella. In some cases, bacterial infections like a middle ear infection and bacterial pneumonia have also been linked. What are the symptoms? Symptoms can start slowly and progressively worsen over hours or days. Damage depends on the affected area of the spinal cord.
    [Show full text]
  • Inflammation of the Central Nervous System- Encephalitis, Myelitis, and Meningitis
    Inflammation of the Central Nervous System- Encephalitis, Myelitis, and Meningitis Stacy Dillard, DVM, Diplomate ACVIM (Neurology) Inflammation of the central nervous system is a very common condition seen in veterinary neurology. The area in which the inflammation predominates indicates which of the clinical syndromes we use to name the disease. Inflammation involving the brain is called encephalitis, of the spinal cord is called myelitis and of the meninges is called meningitis. It is common to have more than one area of the central nervous system affected and therefore combinations of the names are often used such as meningoencephalomyelitis or meningoencephalitis. Encephalitis is the most common area of the central nervous system to be affected and will be used as the general term in this article. Etiology There are two main classes of encephalitis: infectious and idiopathic. Infectious etiologies include viruses, fungi, bacteria, protozoa, tick borne diseases and even algae. True infectious encephalitis is much less common in the dog than in other species including humans; however diagnosis is critical for proper targeted therapy. Idiopathic encephalitis means that an infectious etiology has not been found and encompasses a wide range of syndromes appreciated in the canine patient. Many idiopathic encephalitidies are thought to be immune-mediated and respond well to immune-suppression. Other idiopathic encephalitis, such as necrotizing encephalitis found in young toy breed dogs, do not appear to respond as well to immuno- suppression and therefore may represent another form of the disease. Idiopathic encephalitis is much more common in the dog than infectious encephalitis; however, definitive diagnosis is critical as treatment is markedly different between the two categories of disease.
    [Show full text]
  • Syringomyelia and Arachnoiditis
    106 Journal of Neurology, Neurosurgery, and Psychiatry 1990;53:106-113 Syringomyelia and arachnoiditis L R Caplan, A B Norohna, L L Amico Abstract and weakness in his right hand. Months later, Five patients with chronic arachnoiditis he developed sensory loss and weakness of the and syringomyelia were studied. Three hands, more noticeable in the left than the patients had early life meningitis and right. Atrophy, frequent burns, and hand developed symptoms of syringomyelia tremor were reported by the patient. Four eight, 21, and 23 years after the acute years later, the lower extremities became weak, infection. One patient had a spinal dural initially on the right side. He then experienced thoracic AVM and developed a thoracic weakness in the left leg which soon became syrinx 11 years after spinal subarachnoid more severe than in the right. He complained of haemorrhage and five years after surgery a drawing, burning pain in both arms from the on the AVM. A fifth patient had tuber- hands to the radial forearms and from the hips culous meningitis with transient spinal to the toes. cord dysfunction followed by develop- In 1943 (aged 46), he was first evaluated at ment ofa lumbar syrinx seven years later. the Harvard Neurological Unit at Boston City Arachnoiditis can cause syrinx formation Hospital. There was diminished body hair and by obliterating the spinal vasculature burn scars on his fingers. Mental function and causing ischaemia. Small cystic regions cranial nerves were normal except for a slight of myelomalacia coalesce to form left lower facial droop and slighlt leftward cavities. In other patients, central cord protrusion of the tongue.
    [Show full text]
  • Paraneoplastic Neurological and Muscular Syndromes
    Paraneoplastic neurological and muscular syndromes Short compendium Version 4.5, April 2016 By Finn E. Somnier, M.D., D.Sc. (Med.), copyright ® Department of Autoimmunology and Biomarkers, Statens Serum Institut, Copenhagen, Denmark 30/01/2016, Copyright, Finn E. Somnier, MD., D.S. (Med.) Table of contents PARANEOPLASTIC NEUROLOGICAL SYNDROMES .................................................... 4 DEFINITION, SPECIAL FEATURES, IMMUNE MECHANISMS ................................................................ 4 SHORT INTRODUCTION TO THE IMMUNE SYSTEM .................................................. 7 DIAGNOSTIC STRATEGY ..................................................................................................... 12 THERAPEUTIC CONSIDERATIONS .................................................................................. 18 SYNDROMES OF THE CENTRAL NERVOUS SYSTEM ................................................ 22 MORVAN’S FIBRILLARY CHOREA ................................................................................................ 22 PARANEOPLASTIC CEREBELLAR DEGENERATION (PCD) ...................................................... 24 Anti-Hu syndrome .................................................................................................................. 25 Anti-Yo syndrome ................................................................................................................... 26 Anti-CV2 / CRMP5 syndrome ............................................................................................
    [Show full text]
  • Transverse Myelitis
    TRANSVERSE MYELITIS Demyelinating Central Nervous System Disease WHAT IS TRANSVERSE MYELITIS? Transverse myelitis is an inflammation of both sides of one section of the spinal cord. This neurological disorder often damages the insulating material covering nerve cell fibers (myelin). Transverse myelitis interrupts the messages that the spinal cord nerves send throughout the body. This can cause pain, muscle weakness, paralysis, sensory problems, or bladder and bowel dysfunction. Possible causes of transverse myelitis may include infections and immune system disorders that attack the body's tissues. It could also be caused by other myelin disorders, such as multiple sclerosis. Treatment for transverse myelitis includes medications and rehabilitative therapy. Most people with transverse myelitis recover at least partially. Those with severe attacks sometimes are left with major disabilities. WHAT ARE THE SYMPTOMS? Typical signs and symptoms include: • Pain. Transverse myelitis pain may begin suddenly in your lower back. Sharp pain may shoot down your legs or arms or around your chest or abdomen. Pain symptoms vary based on the part of your spinal cord that's affected. • Abnormal sensations. Some people with transverse myelitis report sensations of numbness, tingling, coldness or burning. Some are especially sensitive to the light touch of clothing or to extreme heat or cold. You may feel as if something is tightly wrapping the skin of your chest, abdomen or legs. • Weakness in your arms or legs. Some people notice that they're stumbling or dragging one foot, or heaviness in the legs. Others may develop severe weakness or even total paralysis. • Bladder and bowel problems. This may include needing to urinate more frequently, urinary incontinence, difficulty urinating and constipation.
    [Show full text]
  • ADEM) After Autologous Peripheral Blood Stem Cell Transplant for Non-Hodgkin’S Lymphoma
    Bone Marrow Transplantation, (1999) 24, 1351–1354 1999 Stockton Press All rights reserved 0268–3369/99 $15.00 http://www.stockton-press.co.uk/bmt Case report Acute disseminated encephalomyelitis (ADEM) after autologous peripheral blood stem cell transplant for non-Hodgkin’s lymphoma A Re and R Giachetti Department of Hematology, University of Parma, Italy Summary: High-dose chemotherapy followed by autologous periph- eral blood stem cell transplantation (PBSCT) is a thera- Acute disseminated encephalomyelitis (ADEM) is a peutic intervention performed with increasing frequency for demyelinating disorder of the central nervous system hematologic and solid malignancies.4 The widespread use with an acute clinical onset and a wide variability in of this procedure depends on its safety and easy feasibility. severity and outcome. It usually follows a viral infection Mild organ toxicities and low incidence of life-threatening or an immunization and is thought to be immuno- complications are usually reported. Neurologic events are mediated. We report a case of ADEM with a dramatic frequently mild and reversible and usually secondary to clinical onset in an autologous peripheral blood stem injury to other organ systems.5 cell transplant (PBSCT) recipient for non-Hodgkin’s We report a case of ADEM in an autologous PBSCT lymphoma who developed the neurologic syndrome 12 recipient with non-Hodgkin’s lymphoma (NHL) who days after PBSC reinfusion. This is the first report of developed the syndrome on day ϩ12 after PBSC ADEM in the setting of autologous PBSCT, a thera- reinfusion, without any recognizable etiology. To our peutic procedure performed with increasing frequency knowledge, this is the first report of ADEM developing in a wide variety of hematologic and solid malignancies.
    [Show full text]
  • Acute Inflammatory Myelopathies
    UCSF UC San Francisco Previously Published Works Title Acute inflammatory myelopathies. Permalink https://escholarship.org/uc/item/3wk5v9h9 Journal Handbook of clinical neurology, 122 ISSN 0072-9752 Author Cree, Bruce AC Publication Date 2014 DOI 10.1016/b978-0-444-52001-2.00027-3 Peer reviewed eScholarship.org Powered by the California Digital Library University of California Handbook of Clinical Neurology, Vol. 122 (3rd series) Multiple Sclerosis and Related Disorders D.S. Goodin, Editor Copyright © 2014 Bruce Cree. Published by Elsevier B.V. All rights reserved Chapter 28 Acute inflammatory myelopathies BRUCE A.C. CREE* Department of Neurology, University of California, San Francisco, USA INTRODUCTION injury caused by the acute inflammation and the likeli- hood of recurrence differs depending on the etiology. Spinal cord inflammation can present with symptoms sim- Additional important diagnostic and prognostic features ilar to those of compressive myelopathies: bilateral weak- include whether the myelitis is partial or transverse, ness and sensory changes below the spinal cord level of febrile illness, the number of vertebral spinal cord injury, often accompanied by bowel and bladder impair- segments involved on MRI at the time of acute attack, ment and sparing cranial nerve and cerebral function. the rapidity from symptom onset to maximum deficit, Because of the widespread availability of magnetic reso- and the severity of involvement. nance imaging (MRI) and computed tomography (CT) imaging, compressive etiologies can be rapidly excluded, METHODOLOGIC CONSIDERATIONS leading to the consideration of non-compressive etiologies for myelopathy. The differential diagnosis of non- Large observational cohort studies or randomized con- compressive myelopathy is broad and includes infectious, trolled trials concerning myelitis have never been under- parainfectious, toxic, nutritional, vascular, and systemic taken.
    [Show full text]
  • Pearls: Myelopathy
    Pearls: Myelopathy Neeraj Kumar, M.D.1 ABSTRACT Both general neurologists and neurologists with a broad spectrum of subspecialty interests are often asked to evaluate patients with disorders of the spinal cord. Over the past decade, there have been significant advances in our understanding of a wide spectrum of immune-mediated, infectious, metabolic, hereditary, paraneoplastic, and compressive myelopathies. Advances have been made in the classification and management of spinal vascular malformations. Aortic reconstruction surgery has led to an increased incidence of spinal cord stroke. It is important to recognize a dural arteriovenous fistula as a cause of progressive myelopathy. In the past, noninfectious inflammatory myelopathies have frequently been categorized as idiopathic transverse myelitis. Advances in neuroimaging and discovery of a serum antibody marker, neuromyelitis optica-immunoglobulin G (NMO-IgG), have allowed more specific diagnoses, such as multiple sclerosis and neuro- myelitis optica. Abnormalities suggestive of demyelinating disease on brain magnetic resonance imaging (MRI) are known to be highly predictive of conversion to multiple sclerosis in a patient who presents with a transverse myelitis (‘‘clinically isolated syn- drome’’). Acquired copper deficiency can cause a clinical picture that mimics the subacute combined degeneration seen with vitamin B12 deficiency. A history of bariatric surgery is commonly noted in patients with copper deficiency myelopathy. Genetics has advanced our understanding of the complex field of hereditary myelopathies. Three hereditary myelop- athy phenotypes are recognized: predominantly cerebellar (e.g., Friedreich’s ataxia), predominantly motor (e.g., hereditary spastic paraparesis), and a leukodystrophy phenotype (e.g., adrenomyeloneuropathy). Evaluation of myelopathies when no abnormalities are seen on spinal cord imaging is a commonly encountered diagnostic challenge.
    [Show full text]