Chronic Neurological Diseases: Subacute Sclerosing Panencephalitis, Progressive Multifocal Leukoencephalopathy, Kuru, Creutzfeldt-Jakob Disease

1976 ◽  
pp. 519-537 ◽  
Author(s):  
Jacob A. Brody ◽  
Clarence Joseph Gibbs
Keyword(s):  
Progressive Multifocal Leukoencephalopathy ◽  
Neurological Diseases ◽  
Subacute Sclerosing Panencephalitis ◽  
Jakob Disease

Antibody Levels to Herpes Simplex Type 1, Measles and Rubella Viruses in Psychiatric Patients

1974 ◽  
Vol 125 (588) ◽  
pp. 461-465 ◽  
Author(s):  
P. E. Halonen ◽  
R. Rimon ◽  
Katve Arohonka ◽  
V. Jäntti
Keyword(s):  
Herpes Simplex ◽  
Neurological Diseases ◽  
Psychiatric Patients ◽  
Subacute Sclerosing Panencephalitis ◽  
Systematic Search ◽  
Brain Cells ◽  
Histopathological Studies ◽  
Antibody Levels ◽  
Infectious Aetiology

The systematic search of aetiological agents from a variety of slowly progressing or subacute neurological diseases has revealed causative viruses or virus-like agents from kuru, Creutzfeldt-Jacob disease and other forms of presenile dementias, subacute sclerosing panencephalitis, progressive multifocal encephalopathy, and from many similar neurological diseases in animals (Gajdusek and Gibbs, 1973; Gajdusek, 1973). The first two diseases called subacute spongiform virus encephalopathies (Gajdusek and Gibbs, 1971) have many interesting features including heredo-familial occurrence and totally non-inflammatory neuropathology. Thus the epidemiology of these diseases is not typical for diseases with infectious aetiology, and the histopathological studies do not suggest the presence of extremely high-titred infectious material in brain cells.

scholarly journals Aids-related progressive multifocal leukoencephalopathy: a retrospective study in a referral center in São Paulo, Brazil

2008 ◽  
Vol 50 (4) ◽  
pp. 209-212 ◽  
Author(s):  
José E. Vidal ◽  
Augusto C. Penalva de Oliveira ◽  
Maria Cristina D. S. Fink ◽  
Cláudio S. Pannuti ◽  
J. Roberto Trujillo
Keyword(s):  
Progressive Multifocal Leukoencephalopathy ◽  
Opportunistic Infections ◽  
Neurological Diseases ◽  
Clinical Manifestations ◽  
Sao Paulo ◽  
São Paulo ◽  
Referral Center ◽  
Acquired Immunodeficiency ◽  
Few Data ◽  
Immunodeficiency Syndrome

Few data are available about progressive multifocal leukoencephalopathy (PML) in patients with acquired immunodeficiency syndrome (AIDS) from Brazil. The objectives of this study were to describe the main features of patients with PML and estimate its frequency among AIDS patients with central nervous system (CNS) opportunistic diseases admitted to the Instituto de Infectologia Emílio Ribas, São Paulo, Brazil, from April 2003 to April 2004. A retrospective and descriptive study was performed. Twelve (6%) cases of PML were identified among 219 patients with neurological diseases. The median age of patients with PML was 36 years and nine (75%) were men. Nine (75%) patients were not on antiretroviral therapy at admission. The most common clinical manifestations were: focal weakness (75%), speech disturbances (58%), visual disturbances (42%), cognitive dysfunction (42%), and impaired coordination (42%). The median CD4+ T-cell count was 45 cells/µL. Eight (67%) of 12 patients were laboratory-confirmed with PML and four (33%) were possible cases. Eleven (92%) presented classic PML and only one case had immune reconstitution inflammatory syndrome (IRIS)-related PML. In four (33%) patients, PML was the first AIDS-defining illness. During hospitalization, three patients (25%) died as a result of nosocomial pneumonia and nine (75%) were discharged to home. Cases of PML were only exceeded by cases of cerebral toxoplasmosis, cryptococcal meningoencephalitis, and CNS tuberculosis, the three more frequent neurologic opportunistic infections in Brazil. The results of this study suggest that PML is not an uncommon HIV-related neurologic disorder in a referral center in Brazil.

Detection and Analysis of Animal Materials in Food and Feed

2000 ◽  
Vol 63 (11) ◽  
pp. 1602-1609 ◽  
Author(s):  
DRAGAN MOMCILOVIC ◽  
AVRAHAM RASOOLY
Keyword(s):  
Animal Feed ◽  
Neurological Diseases ◽  
Transmissible Spongiform Encephalopathies ◽  
Spongiform Encephalopathy ◽  
Variant Form ◽  
Spongiform Encephalopathies ◽  
Food And Feed ◽  
Ruminant Animals ◽  
Jakob Disease ◽  
Animal Material

Bovine spongiform encephalopathy (BSE) belongs to a group of progressively degenerative neurological diseases known as transmissible spongiform encephalopathies (TSEs) associated with a variant form of Creutzfeldt-Jakob disease in humans. TSEs are fatal diseases caused by prions (proteinaceous infectious particle) and are characterized by an incubation period that may range from several months to several years, depending on the host. Because BSE is spread through animal feed, the main strategy for preventing the establishment and spread of BSE is to prohibit the use of proteins derived from mammalian tissue in feed for ruminant animals. Enforcement of these regulations relies on the ability to identify the presence of prohibited proteins in ruminant feed. The methods to detect bovine products in rendered and cooked materials are based on analyses of DNA, bone, or protein. In this article, we discuss the current methodology as well as other potentially useful methods of analysis of animal material in food. While methods are generally useful, none specifically distinguish between prohibited bovine material and allowable bovine products, such as milk or blood. Furthermore, all these methods are hampered by the fact that the rendering process involves heat treatment that denatures and degrades proteins and DNA. There is a need for improving existing methods and developing new methods to overcome these two limitations.

scholarly journals Post-anoxic myoclonus

2014 ◽  
Vol 2 (6) ◽  
pp. 8
Author(s):  
Pavis Laengvejkal ◽  
Parunyou Julayanont ◽  
Drew Payne
Keyword(s):  
Brain Injury ◽  
Clinical Presentation ◽  
Subacute Sclerosing Panencephalitis ◽  
Chronic Phase ◽  
Muscular Contraction ◽  
Comorbid Conditions ◽  
Anoxic Brain Injury ◽  
Hypoxic Event ◽  
Negative Myoclonus ◽  
Jakob Disease

Myoclonus is a movement disorder characterized by involuntary, sudden, brief muscle jerks caused by muscular contraction (positive myoclonus) or inhibition (negative myoclonus).1,2 Myoclonus is generally a medical sign and not a diagnosis. It can occur in multiple disorders. A short differential diagnosis list includes anoxic brain injury, multiple sclerosis, Parkinson's disease, subacute sclerosing panencephalitis, and Creutzfeldt-Jakob disease.  One way to classify the etiologies is through review of the clinical presentation and comorbid conditions. This article presents a review of anoxic brain injury related myoclonus. Post-anoxic myoclonus (PAM) can develop in either acute or chronic phase. Acute PAM occurs within hours after hypoxic event; chronic PAM (Lance-Adams syndrome) develops in survivors several days to weeks after the episodes of brain hypoxia.

Palliative care in non-malignant neurological disease

2019 ◽  
pp. 619-636
Keyword(s):  
Parkinson’S Disease ◽  
Palliative Care ◽  
Parkinson's Disease ◽  
Multiple System Atrophy ◽  
Neurodegenerative Disorder ◽  
Neurological Diseases ◽  
Urogenital Dysfunction ◽  
Jakob Disease ◽  
The Central Nervous System ◽  
Incomplete Bladder Emptying

This chapter covers the palliative care aspects of non-malignant neurological diseases, including multiple sclerosis, Parkinson’s disease, motor neurone disease, multiple system atrophy, progressive supranuclear palsy, and Creutzfeldt–Jakob disease. MS is a disease characterized by inflammation and demyelination affecting the central nervous system and ultimately injury and gliosis. Parkinson’s disease (PD) is the commonest neurodegenerative disease after Alzheimer’s disease, with an estimated incidence of 2/1000. It affects just under 1% of people over the age of 65 years. PD is probably not one disease but several with common clinical features. Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by Parkinsonian features, plus autonomic dysfunction in the form of orthostatic hypotension, and/or urogenital dysfunction in the form of incontinence and incomplete bladder emptying. At times it can also include cerebellar symptoms. It is not hereditary, and affects adults usually in the fourth or fifth decade. Post-mortem studies of patients diagnosed with PD indicate that 10–25% had multiple system atrophy.

scholarly journals Syndromes of Rapidly Progressive Cognitive Decline-Our Experience

2017 ◽  
Vol 08 (S 01) ◽  
pp. S66-S71 ◽  
Author(s):  
Sadanandavalli Retnaswami Chandra ◽  
Lakshminarayanapuram Gopal Viswanathan ◽  
Anupama Ramakanth Pai ◽  
Rahul Wahatule ◽  
Suvarna Alladi
Keyword(s):  
Small Vessel Disease ◽  
Subacute Sclerosing Panencephalitis ◽  
Treatment Options ◽  
Vitamin B12 Deficiency ◽  
Autoimmune Encephalitis ◽  
Acid Decarboxylase ◽  
Thyroid Peroxidase ◽  
Immune Mediated ◽  
Thyroid Peroxidase Antibody ◽  
Jakob Disease

ABSTRACT Background: Dementias are fairly slowly progressive degenerative diseases of brain for which treatment options are very less and carry a lot of burden on family and society. A small percentage of them are rapidly progressive and mostly carry a different course outcome. However, there are no definite criteria other than the time line for these patients. Aims: The aim of this was to identify and categorize the causes and course of rapidly progressive dementias seen in our center. Settings and Design: Patients who presented with rapid deterioration of cognitive functions within weeks to 1 year between 2011 and December 2016 were evaluated. Patients and Methods: All patients underwent all mandatory tests for dementia including brain imaging. Complete vasculitis workup, autoimmune encephalitis profile including Voltage Gated Potassium Channel, N-methyl-D-aspartic acid receptor, glutamic acid-decarboxylase, thyroid-peroxidase antibody, cerebrospinal fluid, and other special tests such as duodenal biopsy and paraneoplastic workup were done based on clinical indications. Results and Conclusions: Out of 144 patients 42 had immune-mediated encephalopathy, 18 had Creutzfeldt-Jakob disease, 3 had Vitamin B12 deficiency, 63 had infection with neurocysticercosis, 7 had tuberculosis, 2 had HIV, 1 had herpes simplex encephalitis, 1 had neurosyphilis, 1 Whipples disease, 1 had Subacute Sclerosing Panencephalitis, 1 had Mass lesion, 3 had Frontotemporal dementia, and 3 had small vessel disease. Good majority of these patients have infective and immune-mediated causes and less number belong to degenerative group. Therefore, caution is needed to look for treatable cause as it carries a different treatment options and outcome.

The Neurophysiological Basis of Myoclonus

2017 ◽  
Author(s):  
Phillip D. Thompson ◽  
Hiroshi Shibasaki ◽  
Mark Hallett
Keyword(s):  
Subacute Sclerosing Panencephalitis ◽  
Sensory Stimuli ◽  
Classification Schemes ◽  
Clinical Appearance ◽  
Cortical Myoclonus ◽  
Somatosensory Evoked Response ◽  
Spinal Myoclonus ◽  
Jakob Disease ◽  
Neurophysiological Assessment ◽  
Neurophysiological Basis

There are several types of myoclonus, with a variety of classification schemes, and the clinician must determine what type of myoclonus a patient has and what type of neurophysiological assessment can facilitate diagnosis. The electromyographic (EMG) correlate of the myoclonus should be examined, including the response to sensory stimuli (C-reflex). The electroencephalographic (EEG) correlate of the myoclonus should then be examined, possibly including back-averaging from the myoclonus or looking at corticomuscular (EEG–EMG) coherence. The somatosensory evoked response (SEP) should be obtained. Such studies will help determine the myoclonus origin, most commonly cortical or brainstem. One form of cortical myoclonus has the clinical appearance of a tremor (cortical tremor). Brainstem myoclonus includes exaggerated startle (hyperekplexia). Other forms of myoclonus include spinal myoclonus and functional myoclonus, which have their own distinct physiological signature. Several causes of myoclonus are reviewed, including rare types such as Creutzfeldt-Jakob disease and subacute sclerosing panencephalitis.

Viral infections of the nervous system

1984 ◽  
Vol 61 (2) ◽  
pp. 207-224 ◽  
Author(s):  
Leslie P. Weiner ◽  
John O. Fleming
Keyword(s):  
Viral Infections ◽  
Current Knowledge ◽  
Subacute Sclerosing Panencephalitis ◽  
Epidemic Encephalitis ◽  
Laboratory Findings ◽  
Spongiform Encephalopathies ◽  
Content Type ◽  
Neurotropic Viruses ◽  
Jakob Disease ◽  
Immunodeficiency Syndrome

✓ Neurotropic viruses cause a number of important infectious syndromes including encephalitis, myelitis, meningitis, and radiculopathy. In this review, the biology of conventional and unconventional viruses is examined. The host immune response to viruses is discussed, and patterns of viral pathogenesis are explained. The clinical features, laboratory findings, management of important viral infections, such as herpes simplex encephalitis and epidemic encephalitis, are presented. Post-infection syndromes, such as the Guillain-Barré syndrome, and chronic viral infections, such as those causing progressive multifocal leukoencephalopathy and subacute sclerosing panencephalitis, are discussed. Current knowledge concerning the nature of unconventional virus-like agents of the spongiform encephalopathies, including kuru and Creutzfeldt-Jakob disease, is summarized. Finally, viral infections of immunocompromised patients and the possible role of viruses in the newly described acquired immunodeficiency syndrome (AIDS) are examined.

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