Acquired hepatocerebral degeneration and hepatic encephalopathy: correlations and variety of clinical presentations in overt and subclinical liver disease

Abstract

Acquired hepatocerebral degeneration (AHD) and hepatolenticular degeneration can have similar clinical presentations, but when a chronic liver disease and atypical motor findings coexist, the distinction between AHD and hepatic encephalopathy (HE) can be even more complicated. We describe three cases of AHD (two having HE) with different neuroimaging findings, distinct hepatic diseases and similar motor presentations, all presenting chronic arterial hypertension and weight loss before the disease manifestations. The diagnosis and physiopathology are commented upon and compared with previous reports. In conclusion, there are many correlations among HE, hepatolenticular degeneration and AHD, but the overlapping of AHD and HE could be more common depending on the clinical knowledge and diagnostic criteria adopted for each condition. Since AHD is not considered a priority that affects the liver transplant list, the prognosis in AHD patients remains poor, and flow interruption in portosystemic shunts must always be taken into account. Key words: athetosis, chorea, dyskinesias, dystonia, hepatic encephalopathy, hepatolenticular degeneration, hyperkinesis, movement disorders.Degeneração hepatocerebral adquirida e encefalopatia hepática: correlações e variedade de apresentações clínicas na doença hepática evidente e subclínica RESUMO A degeneração hepatocerebral adquirida (AHD) e a degeneração hepatolenticular podem ter apresentações clínicas semelhantes, mas quando uma doença hepática crônica e achados motores atípicos coexistem, a distinção entre AHD e encefalopatia hepática (HE) pode ser ainda mais complicada. Descrevemos três casos de AHD (dois tendo HE) com diferentes achados em neuroimagem, doenças hepáticas distintas e apresentações motoras semelhantes, todos com hipertensão arterial e perda de peso antes das manifestações motoras. O diagnóstico e a fisiopatologia são comentados e comparados com relatos prévios. Concluímos que existem muitas correlações entre HE, degeneração hepatolenticular e AHD, mas a sobreposição de HE e AHD pode ser mais comum dependendo do conhecimento clínico e da acurácia dos critérios diagnósticos adotados para cada enfermidade. Como a AHD não é considerada prioridade na lista de transplante hepático, o prognóstico dos pacientes com AHD permanece ruim, e a interrupção do fluxo nos shunts portossistêmicos deve ser sempre considerada.

Acquired hepatocerebral degeneration

“…Typical MR findings show an abnormally high signal on T1-weighted imaging in the basal ganglia, particularly globus pallidus. This high signal is now believed to be due to manganese deposition, and postmortem studies have shown levels up to 7 times normal in globus pallidus confirming the main role of manganese in the pathogenesis of the disease 8. Magnetic resonance imaging findings of our patient were typical for AHD.…”

“…The second form is referred to as a syndrome of neuropsychiatric, neuropsychological and neurological disturbances arising as a complication of liver disease. Despite previous studies suggesting the condition is reversible, there is growing evidence that reversibility is incomplete 7,8…”

“…The first one, which used to be referred to as latent or subclinical HE according to the old terminology, is now replaced with the term minimal HE. It is characterized by mild impairment of visuospatial function, attention and psychomotor speed 7,8. The second form is referred to as a syndrome of neuropsychiatric, neuropsychological and neurological disturbances arising as a complication of liver disease.…”

Acquired hepatocerebral degeneration

“…MRI changes have been reported in cases of acute hepatic encephalopathy; however, such cases respond to medical therapy 6. It is difficult to differentiate between neurological symptoms owing to hyperammonaemia which occurs in the case of hepatic encephalopathy or owing to acquired hepatocerebral degeneration, as pathogenesis of both these entities is complex and not fully understood 7. Our patient despite having high levels of ammonia did not respond to medical management for hepatic encephalopathy and there was no consciousness impairment, both features are present in hepatic encephalopathy.…”

Edinsel Hepatoserebral Dejenerasyon: Olgu sunumu

“…Mümkün olursa karaciğer transplantasyonu yapılabilir. Nörolojik semptomların tedavisinde mevcut bulgulara göre dopamin agonistleri, levodopa, ya da dopamin antagonistleri, tetrabenazin verilebilir (3,8).…”