scholarly journals Liver Transplantation Prevents Progressive Neurological Impairment in Argininemia

2013 ◽  
pp. 25-30 ◽  
Author(s):  
E. Santos Silva ◽  
M. L. Cardoso ◽  
L. Vilarinho ◽  
M. Medina ◽  
C. Barbot ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Neurological Impairment

Moyamoya disease as a possible cause of neurological impairment following liver transplantation for Wilson's disease

2011 ◽  
Vol 41 (3) ◽  
pp. 282-286 ◽  
Author(s):  
Federico Desideri ◽  
Massimo Marignani ◽  
Olivier Vanovermeire ◽  
Isabelle Colle ◽  
Gianfranco Delle Fave ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Moyamoya Disease ◽  
Wilson’S Disease ◽  
Neurological Impairment ◽  
Wilson's Disease ◽  
Possible Cause

Liver Transplantation in Wilson's Disease with Neurological Impairment: Evaluation in 4 Patients

2016 ◽  
Vol 77 (1-2) ◽  
pp. 5-15 ◽  
Author(s):  
Chloé Laurencin ◽  
Anne Sophie Brunet ◽  
Jérôme Dumortier ◽  
Laurence Lion-Francois ◽  
Stéphane Thobois ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Wilson’S Disease ◽  
Neurological Impairment ◽  
Wilson's Disease

scholarly journals Liver Transplantation for Neuropsychiatric Wilson Disease

1998 ◽  
Vol 12 (1) ◽  
pp. 65-68 ◽  
Author(s):  
Narmin Kassam ◽  
Norbert Witt ◽  
Norman Kneteman ◽  
Vincent G Bain
Keyword(s):  
Liver Transplantation ◽  
Wilson Disease ◽  
English Language ◽  
Neurological Impairment ◽  
Neurological Recovery ◽  
Post Transplantation ◽  
Neuropsychiatric Manifestations ◽  
Aggressive Medical Management ◽  
Psychiatric Manifestations ◽  
Language Literature

Although neuropsychiatric manifestations are prominent in some patients with Wilson disease, there is little published information regarding the efficacy of liver transplantation for these patients. A 22-year-old male with advanced neurological impairment and prominent psychiatric manifestations due to Wilson disease who underwent liver transplantation is presented. After transplantation, the ceruloplasmin and copper studies normalized and eventually the Kayser-Fleischer rings disappeared. Neurological recovery was very slow and incomplete, and his behavioural and personality disorder was entirely unaffected. He committed suicide 43 months post-transplantation. A review of the small number of related published cases in the English language literature shows variable neurological recovery post-transplantation, but the course of psychiatric manifestations is virtually never described. This case suggests that one must be cautious regarding liver transplantation for Wilson disease in patients with prior psychiatric manifestations. Aggressive medical management is likely to be preferable in most cases.

scholarly journals Liver-transplantation-corrected coagulation dysfunction and neurological impairment in patients with argininemia

2020 ◽  
Author(s):  
Bin Cui ◽  
Li-Ying Sun ◽  
Lin Wei ◽  
Wei Qu ◽  
Zhi-Gui Zeng ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Failure To Thrive ◽  
Neurological Impairment ◽  
Ammonia Concentration ◽  
Morphological Examination ◽  
Neurodevelopmental Outcomes ◽  
Inborn Errors ◽  
Number Of Patients ◽  
Friendship Hospital ◽  
Coagulation Dysfunction

Abstract Background Argininemia is a rare disease caused by inborn errors of metabolism. Advances in diagnosis and treatment have increased the number of patients receiving effective management. Argininemia is characterized by progressive spastic paraplegia, mental retardation, failure to thrive and irritability, and patients with coagulopathy are paid more attention. We studied the changes in coagulation dysfunctions and neurodevelopment in patients with argininemia before and after liver transplantation(LT). Methods The data in this study were obtained from the Liver Transplantation Center of Beijing Friendship Hospital, Capital Medical University between January 2015 and November 2019. We identified data related to argininemia in all patients diagnosed with urea cycle disorder, and extracted the details on coagulation, liver function, neurodevelopmental outcomes, histopathological and morphological examination, and other clinical presentations. The patients were followed up by telephone and/or in the clinic. Results Nine patients with argininemia diagnosed by tandem mass spectrometry and symptoms, and confirmed by gene sequencing. The symptoms deteriorated after dietary restriction in all patients with argininemia. Coagulopathy manifested before surgery, and no significant correlation was detected with plasma ammonia concentration. The coagulation dysfunction was completely resolved and progressive neurological impairment was prevented in seven patients with LT. Conclusions Coagulation dysfunctions are common manifestations of patients with

scholarly journals Brain metastasis of hepatocellular carcinoma detected after liver transplantation

2004 ◽  
Vol 41 (3) ◽  
pp. 199-201 ◽  
Author(s):  
Alex Vianey Callado França ◽  
Ana Martinelli ◽  
Orlando Castro e Silva Jr.
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Transplantation ◽  
Brain Metastasis ◽  
Arterial Embolization ◽  
Neurological Impairment ◽  
Hepatitis C Infection ◽  
Histologic Diagnosis ◽  
Ethanol Injection ◽  
Transplant Patients ◽  
Hepatic Lesions

AIM: We report the case of a patient with hepatocellular carcinoma submitted to liver transplantation, who subsequently manifested tumor recurrence initially as brain metastasis. CASE DESCRIPTION: A 48-year-old male cirrhotic patient with hepatitis C infection, and two focal hepatic lesions, had a cytologic and histologic diagnosis of hepatocellular carcinoma. Before transplant, he was submitted to adjuvant treatment with a combination of arterial embolization and intratumoral ethanol injection. In the 3rd month post-liver transplantation, the patient developed headache, nausea and vomiting, without any neurological impairment. Brain computed tomography and magnetic resonance imaging identified an expansive hypervascular lesion with internal bleeding. Evaluation of the surgical explant revealed macroscopic invasion of portal vessels. CONCLUSION: Brain metastasis of a hepatocellular carcinoma after liver transplantation may occur. This metastasis may have occurred before or soon after the transplant. Patients with hepatocellular carcinoma, awaiting liver transplant, should be screened for cerebral metastasis. Vascular invasion may indicate hematogenic dissemination of the tumor.

Central Nerve System Impairment, AMA Guides, Sixth Edition: An Overview

2018 ◽  
Vol 23 (1) ◽  
pp. 10-13
Author(s):  
James B. Talmage ◽  
Jay Blaisdell
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Neurological Impairment ◽  
Sixth Edition ◽  
Central Nerve System ◽  
The Central Nervous System ◽  
The One ◽  
Nerve System ◽  
The Brain

Abstract Injuries that affect the central nervous system (CNS) can be catastrophic because they involve the brain or spinal cord, and determining the underlying clinical cause of impairment is essential in using the AMA Guides to the Evaluation of Permanent Impairment (AMA Guides), in part because the AMA Guides addresses neurological impairment in several chapters. Unlike the musculoskeletal chapters, Chapter 13, The Central and Peripheral Nervous System, does not use grades, grade modifiers, and a net adjustment formula; rather the chapter uses an approach that is similar to that in prior editions of the AMA Guides. The following steps can be used to perform a CNS rating: 1) evaluate all four major categories of cerebral impairment, and choose the one that is most severe; 2) rate the single most severe cerebral impairment of the four major categories; 3) rate all other impairments that are due to neurogenic problems; and 4) combine the rating of the single most severe category of cerebral impairment with the ratings of all other impairments. Because some neurological dysfunctions are rated elsewhere in the AMA Guides, Sixth Edition, the evaluator may consult Table 13-1 to verify the appropriate chapter to use.

Impairment Tutorial: Neurological Impairment Evaluation: Overview of Sixth Edition Approaches

2008 ◽  
Vol 13 (2) ◽  
pp. 6-8
Author(s):  
Lorne Direnfeld ◽  
Christopher R. Brigham ◽  
Elizabeth Genovese
Keyword(s):  
Spinal Cord ◽  
Neurological Impairment ◽  
Diagnostic Techniques ◽  
Neurological Deficits ◽  
Sixth Edition ◽  
Pain Disability ◽  
Whole Person ◽  
Disability Questionnaire ◽  
Cervical Plating

Abstract The AMA Guides to the Evaluation of Permanent Impairment (AMA Guides), does not provide a Diagnosis-based estimate of impairment due to syringomyelia, a disorder in which a cyst (syrinx), develops within the central spinal cord and destroys neural tissue as it expands. The AMA Guides, however, does provide an approach to rating a syringomyelia based on objective findings of neurological deficits identified during a neurological examination and demonstrated by standard diagnostic techniques. Syringomelia may occur after spinal cord trauma, including a contusion of the cord. A case study illustrates the rating process: The case patient is a 46-year-old male who fell backwards, landing on his upper back and head; over a five-year period he received a T5-6 laminectomy and later partial corpectomies of C5, C6, and C7, cervical discectomy C5-6 and C6-7; iliac crest strut graft fusion of C5-6 and C6-7; and anterior cervical plating of C5 to C7 for treatment of myelopathy; postoperatively, the patient developed dysphagia. The evaluating physician should determine which conditions are ratable, rate each of these components, and combine the resulting whole person impairments without omission or duplication of a ratable impairment. The article includes a pain disability questionnaire that can be used in conjunction with evaluations conducted according to Chapter 3, Pain, and Chapter 17, The Spine.

Risk factors for mortality on the liver waiting list of cirrhotic patients listed for liver transplantation

2001 ◽  
Vol 120 (5) ◽  
pp. A77-A77
Author(s):  
D DELPHINE ◽  
F AGNESE ◽  
B NADINE ◽  
L OLIVIER ◽  
L HUBERT ◽  
...  
Keyword(s):  
Risk Factors ◽  
Liver Transplantation ◽  
Waiting List ◽  
Cirrhotic Patients
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