Are Acquired Hepatocerebral Degeneration and Hepatic Myelopathy Reversible?

Pınarbaşı, Binnur; Kaymakoğlu, Sabahattin; Matur, Zeliha; Akyüz, Filiz; Demır, Kadir; Beşışık, Fatih; Özdil, Sadakat; Boztaş, Güngör; Çakalǒglu, Yılmaz; Mungan, Zeynel; Ökten, Atilla

doi:10.1097/mcg.0b013e318150d399

Cited by 54 publications

(64 citation statements)

References 18 publications

(13 reference statements)

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“…Although the cerebral deposition of manganese may have a role in the etiopathogenesis of AHD, there was no report in HM. 18 Though various hypotheses were suggested, the above seems to gain agreement consistently. However, the possibility of a deficiency of a needed liver-synthesized material cannot be excluded.…”

Section: Pathophysiologymentioning

confidence: 99%

“…15,18 In addition to spastic paraparesis or quadriparesis, 6 cases (8.2%) manifested superficial sensation deficit, In a majority of the cases (54/67, 80.6%), plasma ammonia concentrations were found to be elevated. The spinal cord MRI imaging shows no abnormality.…”

Section: Characteristicmentioning

confidence: 99%

“…The spinal cord MRI imaging shows no abnormality. MRI of the brain showed high signal intensity on T1-weighted sequences within the basal ganglia including pallidum, lentiform nucleus, putamen, internal capsule and extensive white matter disease on T2-weighted sequences 7,18 in 21 cases (46.7%).…”

Section: Characteristicmentioning

confidence: 99%

“…To date, there were only 15 cases undergone OLT in the English literature, altogether 11 cases showed quite impressive improvement, 18,21,[24][25][26] mostly recovered to be ambulatory.…”

Section: Characteristicmentioning

confidence: 99%

“…This may be due to the difficulty in finding some hiding collateral circulation such as the retroperitoneal varices. 18 Patients with AHD or HM should initially be evaluated for the presence of portosystemic shunts.…”

Section: Pathophysiologymentioning

confidence: 99%

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Hepatic Myelopathy: Case Report and Review of the Literature

Liao¹,

Yan²,

Wei³

et al. 2015

Liver Res Open J

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Background: Hepatic Myelopathy (HM) is a rare complication of chronic liver disease usually associated with extensive portosystemic shunt of blood, which has been created surgically or has occurred spontaneously, causing progressive spastic paraparesis. Some single cases or short clinical reports describing patients suffering from HM have been published worldwide, but are often scattered. Material and method: One additional case of HM with typical symptoms was presented, and a retrospective survey of the literature in a manner of comprehensive review was undertaken. Results: 46 case reports with 98 patients of HM including ours have been eligibly selected. General information on all cases was summarized. Detailed analysis of the clinical characteristics of HM patients was undertaken. Conclusion: Liver cirrhosis caused by hepatitis B infection and alcoholism is the most frequent causes of HM. Portosystemic shunt which resulted in chronic exposure to toxic substances bypassing the liver play an important role in the pathogenesis. The pathology study consistently disclosed a selective and symmetrical severe loss of myelin in both lateral pyramidal tracts. The predominant neurologic abnormality of HM is the progressive spasticity and weakness in the lower extremities. A typical manifestations, such as triparesis or quadriparesis, sensory deficit, urinary or bladder incontinence and non-pyramidal manifestations such as dysarthria, tremor and ataxia can also occur, which render the disorder more complicated to be diagnosed. Plasma ammonia concentrations were frequently found to be elevated. MEP provides evidence of the early diagnosis of HM and assesses different degrees of neurological involvement. The spinal cord MRI imaging shows no abnormality. Abnormalities of brain magnetic resonance combined with syndrome of brain dysfunction, hepatocerebral degeneration should be taken into consideration. Appending case studies suggest that liver transplantation is a promising therapeutic strategy.

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Section: Pathophysiologymentioning

confidence: 99%

Section: Characteristicmentioning

confidence: 99%

Section: Characteristicmentioning

confidence: 99%

“…To date, there were only 15 cases undergone OLT in the English literature, altogether 11 cases showed quite impressive improvement, 18,21,[24][25][26] mostly recovered to be ambulatory.…”

Section: Characteristicmentioning

confidence: 99%

Section: Pathophysiologymentioning

confidence: 99%

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