Book contents
- Insights into Clinical Neurology
- Insights into Clinical Neurology
- Copyright page
- Dedication
- Contents
- Preface
- Acknowledgments
- Chapter 1 The Amnesic Syndrome
- Chapter 2 Aphasia, Apraxia, and Agnosia
- Chapter 3 Spurious Symptoms and Behaviors
- Chapter 4 Hallucinations
- Chapter 5 Grasping and the Grasp Reflex
- Chapter 6 Asterixis
- Chapter 7 Discussion of the Upper Motor Neuron Syndrome
- Chapter 8 Some Aspects of the Lower Motor Neuron Syndrome
- Chapter 9 The Limbic System
- Chapter 10 Vibration Sensation, the Posterior Columns, and Related Topics
- Chapter 11 Transneuronal Effects
- Chapter 12 Spinal Nerve Roots
- Chapter 13 Ischemic Hypoxic Encephalopathy
- Chapter 14 The Liver–Brain Relationship in Wilson Disease
- Chapter 15 Wernicke Disease
- Chapter 16 Delayed Neurologic Disorders after Toxic or Metabolic Insults
- Chapter 17 Transolfactory Spread in the Pathogenesis of Meningoencephalitis
- Chapter 18 Neurologic Disorders in Endocarditis
- Chapter 19 Secondary Abscess Formation in Primary Brain Lesions
- Chapter 20 Progressive Multifocal Leukoencephalopathy: The Early Years
- Index
- References
Chapter 14 - The Liver–Brain Relationship in Wilson Disease
Published online by Cambridge University Press: 27 July 2023
Book contents
- Insights into Clinical Neurology
- Insights into Clinical Neurology
- Copyright page
- Dedication
- Contents
- Preface
- Acknowledgments
- Chapter 1 The Amnesic Syndrome
- Chapter 2 Aphasia, Apraxia, and Agnosia
- Chapter 3 Spurious Symptoms and Behaviors
- Chapter 4 Hallucinations
- Chapter 5 Grasping and the Grasp Reflex
- Chapter 6 Asterixis
- Chapter 7 Discussion of the Upper Motor Neuron Syndrome
- Chapter 8 Some Aspects of the Lower Motor Neuron Syndrome
- Chapter 9 The Limbic System
- Chapter 10 Vibration Sensation, the Posterior Columns, and Related Topics
- Chapter 11 Transneuronal Effects
- Chapter 12 Spinal Nerve Roots
- Chapter 13 Ischemic Hypoxic Encephalopathy
- Chapter 14 The Liver–Brain Relationship in Wilson Disease
- Chapter 15 Wernicke Disease
- Chapter 16 Delayed Neurologic Disorders after Toxic or Metabolic Insults
- Chapter 17 Transolfactory Spread in the Pathogenesis of Meningoencephalitis
- Chapter 18 Neurologic Disorders in Endocarditis
- Chapter 19 Secondary Abscess Formation in Primary Brain Lesions
- Chapter 20 Progressive Multifocal Leukoencephalopathy: The Early Years
- Index
- References
Summary
The neuropathology of Wilson disease resembles that of chronic nonfamilial hepatocerebral disease. The neurological features of the hereditary disease also resemble those of the nonfamilial disease. Liver transplantation improves both the lesions on MRI scans and the neurologic disorder of Wilson disease. For these reasons and others there is no reason to invoke copper toxicity in the pathogenesis of Wilsonian hepatocerebral disease.
- Type
- Chapter
- Information
- Insights into Clinical Neurology , pp. 72 - 74Publisher: Cambridge University PressPrint publication year: 2023
References
Schiff, E. R., Sorrell, M. F., Maddrey, W. C.. Schiff’s Diseases of the Liver. Chichester, John Wiley & Sons, 2012.Google Scholar
Victor, M., Adams, R. D., Cole, M.. The acquired (non-Wilsonian) type of chronic hepatocerebral degeneration. Medicine (Baltimore) 1965; 44: 345–396.CrossRefGoogle ScholarPubMed
Waggoner, R. W., Malamud, N.. Wilson’s disease in the light of cerebral changes following ordinary acquired liver disorders. J Nerv Ment Dis 1942; 96: 410–423.Google Scholar
Victor, M.. Neurologic changes in liver disease. Res Publ Assoc Res Nerv Ment Dis 1974; 53: 1–12.Google ScholarPubMed
Dusek, P., Litwin, T., Czlonkowska, A.. Neurologic impairment in Wilson disease. Ann Transl Med 2019; 7: S64.CrossRefGoogle ScholarPubMed
Poujois, A., Sobesky, R., Meissner, W. G. et al. Liver transplantation as a rescue therapy for severe neurologic forms of Wilson disease. Neurology 2020; 94: e2189–e2202.CrossRefGoogle ScholarPubMed
Compston, A.. Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver, by Kinnier, S. A. Wilson, (from the National Hospital, and the Laboratory of the National Hospital, Queen Square, London) Brain 1912: 34; 295–509. Brain 2009; 132: 1997–2001.Google Scholar
Shulman, L. M., Minagar, A., Weiner, W. J.. Reversal of parkinsonism following liver transplantation. Neurology 2003; 60: 519.CrossRefGoogle ScholarPubMed
Naegele, T., Grodd, W., Viebahn, R. et al. MR imaging and (1)H spectroscopy of brain metabolites in hepatic encephalopathy: time-course of renormalization after liver transplantation. Radiology 2000; 216: 683–691.CrossRefGoogle ScholarPubMed
Tanner, M. S.. Indian childhood cirrhosis and Tyrolean childhood cirrhosis. Disorders of a copper transport gene? Adv Exp Med Biol 1999; 448: 127–137.CrossRefGoogle ScholarPubMed
Fuentealba, I. C., Aburto, E. M.. Animal models of copper-associated liver disease. Comp Hepatol 2003; 2: 5.CrossRefGoogle ScholarPubMed