scholarly journals Creutzfeldt-Jakob disease: a survey of 14 patients

1996 ◽  
Vol 54 (4) ◽  
pp. 577-583 ◽  
Author(s):  
Paulo E. Marchiori ◽  
Noboru Yasuda ◽  
Helga C. A. Azevedo ◽  
Mônica Órfão ◽  
Dagoberto Callegaro ◽  
...  
Keyword(s):  
Nervous System ◽  
Gamma Globulin ◽  
Clinical Findings ◽  
Status Spongiosus ◽  
Eeg Activity ◽  
School Of Medicine ◽  
Jakob Disease ◽  
The Central Nervous System ◽  
Behaviour Changes ◽  
Visual Disturbances

Creutzfeldt-Jakob disease (CJD) is a transmissible disease of the nervous system causatively related to the presence of an abnormal prion protein, with dementia, myoclonic jerks, and periodic EEG activity. Fourteen patients (7 females and 7 males) ranging from 26 to 76 years of age (median 59 years) were evaluated between 1974 and 1995 at the Neurologic Clinic of São Paulo University School of Medicine. The average duration of the disease was 12 months (3.5 - 34 months). Early clinical findings were: behaviour changes in 7 patients, dementia in 4, visual disturbances in 4, vertigo in 2, tremor in 9, and dystonia in one. Advanced symptoms were dementia and myoclonus in all patients. Pyramidal tract dysfunction was found in 6, cerebellar ataxia in 2, seizures in 3, nystagmus and vertigo in 4, and peripheral nervous system involvement in 2. Atypical clinical forms were found in 5 patients. Periodic EEG activity was found in 10 patients. Cerebrospinal fluid evaluation showed pleocytosis in 1 patient, higher protein content in 2, and higher gamma globulin level in 2. In 10 patients anatomopathological evidence in the central nervous system confirmed the clinical diagnosis by presenting with status spongiosus. All except one patient presented with the sporadic form of the disease.

scholarly journals Superficial siderosis of the central nervous system is a rare and possibly underdiagnosed disorder

2017 ◽  
Vol 75 (2) ◽  
pp. 92-95 ◽  
Author(s):  
Yara Dadalti Fragoso ◽  
Tarso Adoni ◽  
Joseph Bruno Bidin Brooks ◽  
Sidney Gomes ◽  
Marcus Vinicius Magno Goncalves ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Disease Onset ◽  
Clinical Findings ◽  
Neurological Dysfunction ◽  
Superficial Siderosis ◽  
Blood Products ◽  
The Central Nervous System ◽  
Magnetic Resonance Imaging Mri ◽  
Intermittent Bleeding

ABSTRACT Superficial siderosis (SS) of the central nervous system (CNS) is a rare and possibly underdiagnosed disorder resulting from chronic or intermittent bleeding into the subarachnoid space, leading to deposition of blood products in the subpial layers of the meninges. Magnetic resonance imaging (MRI) shows a characteristic curvilinear pattern of hypointensity on its blood-sensitive sequences. Methods Series of cases collected from Brazilian centers. Results We studied 13 cases of patients presenting with progressive histories of neurological dysfunction caused by SS-CNS. The most frequent clinical findings in these patients were progressive gait ataxia, hearing loss, hyperreflexia and cognitive dysfunction. The diagnoses of SS-CNS were made seven months to 30 years after the disease onset. Conclusion SS-CNS is a rare disease that may remain undiagnosed for long periods. Awareness of this condition is essential for the clinician.

scholarly journals Aspergillosis of the central nervous system in a previously healthy patient that simulated Creutzfeldt-Jakob disease

2016 ◽  
Vol 7 (40) ◽  
pp. 940 ◽  
Author(s):  
SergioV Esparza-Gutiérrez ◽  
Adrián Santana-Ramírez ◽  
Pedro Avila-Rodríguez ◽  
JEugenio Jiménez-Gómez ◽  
Ezequiel Vélez-Gómez ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Healthy Patient ◽  
Jakob Disease ◽  
The Central Nervous System

Acute reversible toxic encephalopathy during capecitabine and oxaliplatin treatment

2017 ◽  
Vol 25 (2) ◽  
pp. 497-501 ◽  
Author(s):  
João Godinho ◽  
Mafalda Casa-Nova ◽  
Teresa Mesquita ◽  
Maria João Baptista ◽  
Francisco Araújo ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Findings ◽  
Neurological Dysfunction ◽  
Toxic Encephalopathy ◽  
Central Nervous System Toxicity ◽  
Cerebellar Dysfunction ◽  
Oxaliplatin Treatment ◽  
Tumor Involvement ◽  
The Central Nervous System

Introduction Capecitabine is a fluoropyrimidine commonly used in the treatment of colorectal cancer which may cause central nervous system toxicity, namely cerebellar dysfunction. Case report We describe a 77-year-old man undergoing adjuvant treatment of colon cancer with capecitabine and oxaliplatin who presented with acute cerebellar ataxia and encephalopathy that progressed to coma. Diagnosis of toxic encephalopathy was made after the exclusion of alternative causes of neurological dysfunction and complete resolution of clinical findings with permanent discontinuation of chemotherapy. Discussion When patients with cancer develop symptoms and signs of central nervous dysfunction, metabolic and infectious causes plus tumor involvement of central nervous system must be sought. However, chemotherapy may also cause toxicity to the central nervous system. Capecitabine is no exception, although cerebellar dysfunction is rarely reported. Conclusion Although rare, capecitabine-induced encephalopathy may be severe and physicians should be aware of this possible side effect.

Primary Angiitis of the Central Nervous System Mimicking Sporadic Creutzfeldt-Jakob Disease

2018 ◽  
Vol 32 (3) ◽  
pp. 258-261
Author(s):  
Dian He ◽  
Gang Cai ◽  
Yan Li ◽  
Qi Liu ◽  
Kang Xiao ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Primary Angiitis ◽  
Jakob Disease ◽  
The Central Nervous System

A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system

2020 ◽  
Vol 12 (6) ◽  
pp. 117-123
Author(s):  
L. V. Lukina ◽  
V. A. Mikhailov ◽  
N. I. Ananyeva ◽  
G. E. Mazo ◽  
L. I. Sitnik ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Langerhans Cell Histiocytosis ◽  
Correct Diagnosis ◽  
Brain Lesion ◽  
Clinical Findings ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Bone Lesions ◽  
The Central Nervous System

Langerhans cell histiocytosis (LCH) is a rare disease with hitherto unknown etiology and pathogenesis. It is extremely rare for clinicians to encounter histiocytic lesions of the central nervous system (CNS); the proportion of cases of which is only 1–4% of all polysystemic and multifocal bone lesions. The paper describes a clinical case of fixation amnesia in a female patient with focal brain lesions in LCH. It depicts the most characteristic clinical features and presents an algorithm for the diagnosis of histiocytic brain lesion. The results of the experimental psychological examination of the patient are considered in detail and the clinical presentations of fixation amnesia are described. There are neuroimaging data showing the lesions in the hypothalamic-pituitary region and temporal bone, which involve the auditory structures. The clinical findings have led to the conclusion that both the clinical and neuroimaging patterns of histiocytic lesions in the CNS are non-specific, which complicates the diagnostic search in LCH. For correct diagnosis and timely treatment, it is necessary to perform a biopsy of the pathological focus, followed by histological and immunohistochemical examination of the material.

scholarly journals Neuroinvasion in Prion Diseases: The Roles of Ascending Neural Infection and Blood Dissemination

2010 ◽  
Vol 2010 ◽  
pp. 1-16 ◽  
Author(s):  
Sílvia Sisó ◽  
Lorenzo González ◽  
Martin Jeffrey
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Prion Protein ◽  
Prion Diseases ◽  
Autonomic Nerves ◽  
Peripheral Organs ◽  
Prion Strains ◽  
New Variant ◽  
Jakob Disease ◽  
The Central Nervous System

Prion disorders are infectious, neurodegenerative diseases that affect humans and animals. Susceptibility to some prion diseases such as kuru or the new variant of Creutzfeldt-Jakob disease in humans and scrapie in sheep and goats is influenced by polymorphisms of the coding region of the prion protein gene, while other prion disorders such as fatal familial insomnia, familial Creutzfeldt-Jakob disease, or Gerstmann-Straussler-Scheinker disease in humans have an underlying inherited genetic basis. Several prion strains have been demonstrated experimentally in rodents and sheep. The progression and pathogenesis of disease is influenced by both genetic differences in the prion protein and prion strain. Some prion diseases only affect the central nervous system whereas others involve the peripheral organs prior to neuroinvasion. Many experiments undertaken in different species and using different prion strains have postulated common pathways of neuroinvasion. It is suggested that prions access the autonomic nerves innervating peripheral organs and tissues to finally reach the central nervous system. We review here published data supporting this view and additional data suggesting that neuroinvasion may concurrently or independently involve the blood vascular system.

scholarly journals Neuroplasticity After Spinal Cord Injury and Training: An Emerging Paradigm Shift in Rehabilitation and Walking Recovery

2006 ◽  
Vol 86 (10) ◽  
pp. 1406-1425 ◽  
Author(s):  
Andrea L Behrman ◽  
Mark G Bowden ◽  
Preeti M Nair
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Nervous System ◽  
Paradigm Shift ◽  
Clinical Findings ◽  
Cord Injury ◽  
Rehabilitation Concepts ◽  
The Central Nervous System ◽  
Walking Recovery ◽  
Physiologically Based Approach

AbstractPhysical rehabilitation after spinal cord injury has been based on the premise that the nervous system is hard-wired and irreparable. Upon this assumption, clinicians have compensated for irremediable sensorimotor deficits using braces, assistive devices, and wheelchairs to achieve upright and seated mobility. Evidence from basic science, however, demonstrates that the central nervous system after injury is malleable and can learn, and this evidence has challenged our current assumptions. The evidence is especially compelling concerning locomotion. The purpose of this perspective article is to summarize the evidence supporting an impending paradigm shift from compensation for deficits to rehabilitation as an agent for walking recovery. A physiologically based approach for the rehabilitation of walking has developed, translating evidence for activity-dependent neuroplasticity after spinal cord injury and the neurobiological control of walking. Advanced by partnerships among neuroscientists, clinicians, and researchers, critical rehabilitation concepts are emerging for activity-based therapy to improve walking recovery, with promising clinical findings.

scholarly journals Creutzfeldt–Jakob Disease with a Five-Year Clinical Course, Multicentric Cerebellar Prion Plaques and Prior History of Biopsy-Proven Primary Angiitis of the Central Nervous System: A Case for Iatrogenic Exposure?

Viruses ◽  
2020 ◽  
Vol 12 (12) ◽  
pp. 1411
Author(s):  
Kristina Jeon ◽  
Jeffrey T. Joseph ◽  
Gerard H. Jansen ◽  
Anne Peterson ◽  
J. David Knox ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Parietal Lobe ◽  
Clinical Course ◽  
Prior History ◽  
System A ◽  
Primary Angiitis ◽  
History Of ◽  
Jakob Disease ◽  
The Central Nervous System

Creutzfeldt–Jakob disease (CJD) is a rapidly progressive neurodegenerative disease that can arise spontaneously, genetically, or be acquired through iatrogenic exposure. Most patients die within a year of symptom onset. It is rare, affecting 1–2 per million per year, and the majority of cases are sporadic. Primary angiitis of the central nervous system (PACNS) is also rare, affecting 2.4 per million per year. We present a case of an unusually long clinical course of CJD, almost five years, which began with symptoms of apraxia. The patient had biopsy-proven PACNS 16 years prior to clinical presentation, and the site of biopsy was the left parietal lobe. Autopsy revealed multicentric prion plaques in the cerebellum, in the setting of normal genetic testing. The presence of plaques in the cerebellum, and prior neurosurgery, raises the possibility of iatrogenic exposure. We present the details of this case, including pathology from the original biopsy and final autopsy, as well as a review of relevant cases in the literature.

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