BACKGROUND: Hepatic myelopathy is a very rare neurological complication of chronic liver disease. Patients habitually present with progressive pure motor spastic paraparesis. This neurological dysfunction is almost always due to cirrhosis and portocaval shunt, either surgical or spontaneous.
METHODS: We report two cases of a 57-year-old man and a 37-year-old woman with progressive spastic paraparesis linked to cirrhosis and portal hypertension. The two patients are of Tunisian origin (north Africa). Magnetic resonance imaging of the spinal cord of two patients was normal, while brain magnetic resonance imaging showed a T2 hypersignals of the pallidums. These signs, in favor of hepatic encephalopathy in the two patients with cirrhosis with isolated progressive spastic paraparesis without bladder or sensory disorders, help to retain the diagnosis of hepatic myelopathy.
CONCLUSIONS: Hepatic myelopathy is a severe and debilitating neurological complication of chronic liver disease. The pathogenesis is misunderstood and seems to be multifactorial, including the selective neurotoxic role both of ammonia and other pathogenic neurotoxins. Usually a pathological brain magnetic resonance imaging showing a hepatic encephalopathy was documented, contrasting with a normal spinal cord magnetic resonance imaging that contributed to diagnosis of hepatic myelopathy. Conservative therapies such as ammonia-lowering measures, diet supplementation, antispastic drugs, and endovascular shunt occlusion show little benefit in improving disease symptoms. Liver transplantation performed at early stage can prevent disease progression and could probably allow for recovery.

Ammonia is considered to be a neurotoxin that affects various physiological pathways, including energy metabolism, mitochondria function, and inflammatory response. Dysfunctions of these pathways contribute to the development of cognitive and executive function impairments. A case of a 67-year-old female patient who presented with unusual features of delirium after spine surgery was reported in this study. The patient initially developed acute hepatitis, and 3 weeks later, liver functions gradually improved. However, the acute onset of cognitive impairment and mild hyperammonemia was found. The patient mainly presented with cognitive deficits and impairment of executive functions without a fluctuating course. Her cognitive impairment was resolved when the serum ammonia level returned to the normal range. Consequently, we considered the diagnosis for this patient was delirium rather than a major neurocognitive disorder (dementia). Thus, the clinical diagnosis for delirium and etiologies are discussed.

We describe the endovascular embolization of a 65-year-old man with chronic hepatic encephalopathy. A contrast-enhanced computed tomography demonstrated a splenorenal shunt and a recanalized paraumbilical vein as a continuous portal shunt connecting the left branch of the portal vein and the right common femoral vein. A 2-session embolization was performed for the splenorenal shunt. First, the transvenous approach was used for coil embolization of the splenorenal shunt. It was difficult to advance the catheter system to the embolization site, and it was unstable during coil placement. Second, the paraumbilical venous approach was used to place additional coils. The catheter system had good maneuverability and easily reached the embolization site. Additionally, the stable system allowed for densely packed additional coil implantations. This report demonstrated the paraumbilical venous approach\'s effectiveness in catheter maneuverability and system stability during coil embolization.

Hepatic myelopathy (HM) is a rare neurological complication in the end stage of many liver diseases and is characterized by bilateral spastic paraparesis without sensory and sphincter dysfunction. It occurs owing to metabolic disorders and central nervous system dysfunction associated with cirrhosis. Without timely and effective clinical intervention, the prognosis of these patients is devastating. Although liver transplantation (LT) is an effective treatment for HM, the prognosis of these patients remains unsatisfactory. Early recognition and diagnosis of this disease are essential for improving patient prognosis. Here, we report a case of hepatitis B virus-associated decompensated cirrhosis with HM. The patient recovered well after LT. We also summarize the clinical characteristics and post-transplant outcomes of 25 patients with HM treated by LT through 2023, including this case.

A female in her 70s underwent right hepatectomy with resection of caudate lobe and extrahepatic bile duct for perihilar cholangiocarcinoma(T2aN0M0, Stage Ⅱ: Biliary Cancer Treatment Regulations, 7th edition). On the 4th postoperative day, the patient had impaired consciousness, which worsened to almost coma on the 5th postoperative day. On the same day, a blood test showed high ammonia level, thus the state was thought to be hepatic encephalopathy. Contrast -enhanced CT on the same day showed thrombus from the main trunk of the portal vein to the remnant left branch, narrowing of the lumen of the vessel. Simultaneously, enlarged portosystemic shunt in the pelvic floor due to portal hypertension induced by the thrombosis. Plasmapheresis was performed, and anticoagulation with sodium heparin and antithrombin Ⅲ were started. Then, the portal vein thrombus was reduced, and encephalopathy was improved. She was discharged from the hospital on postoperative day 48. She was treated with edoxaban as an outpatient, and anticoagulation therapy was terminated after a CT scan 6 months after surgery, which confirmed no recurrence of thrombus. She is now alive without recurrence of thrombus or tumor for about 2 years after the surgery.

Combined oxidative phosphorylation deficiency-1 (COXPD1) resulting from a mutation in the G elongation factor mitochondrial 1 (GFM1) gene is an autosomal recessive multisystem disorder arising from a defect in the mitochondrial oxidative phosphorylation system. Death usually appears in the first weeks or years of lifespan.
We report a male patient with ventriculomegaly diagnosed in the 8th month of pregnancy. The delivery was done by caesarean section and respiratory failure occurred immediately after birth. Hypoglycemia, lactic acidosis, elevated gamma-glutamyl transferase and hepatomegaly were confirmed. The brain MRI detected hypoplasia of the cerebellar hemispheres, dilated lateral ventricles, and markedly immature brain parenchyma. Epilepsy had been present since the third month. At 5 months of age, neurological follow-up showed his head circumference to be 37 cm, with plagiocephaly, a low hairline, a short neck, axial hypotonia and he did not adopt any developmental milestones. A genetic mutation, a missense variant in the GFM1 gene, was confirmed: c.748C > T (p.Arg250Trp) was homozygous in the GFM1 gene.
To the best of our knowledge, 28 cases of COXPD1 disease caused by mutations in the GFM1 gene have been described in the literature. COXPD1 should be considered due to symptoms and signs which begin during intrauterine life or at birth. Signs of impaired energy metabolism should indicate that the disease is in the group of metabolic encephalopathies.

BACKGROUND: Acute hyperammonemic encephalopathy is associated with distinct brain MRI findings, namely, hyperintensity in T2-weighted sequences as well as restricted diffusion in diffusion-weighted imaging with accentuation in the insular cortex and cingulate gyrus. The pathophysiology and the histopathological correlates of these characteristic MRI findings are largely unknown.
METHODS: We present a 57-year-old male with a history of chronic alcohol abuse, liver cirrhosis and portal hypertension, and a clinical syndrome (variceal bleeding, depression of consciousness, seizures), elevated plasma ammonia levels, and characteristic brain MRI abnormalities suggestive of acute hyperammonemic encephalopathy. A postmortem histopathological examination revealed extensive hypoxic ischemic encephalopathy without evidence for metabolic encephalopathy. No episodes of prolonged cerebral hypoxemia were documented throughout the course of the disease. We conducted a review of the literature, which exhibited no reports of hyperammonemic encephalopathy in association with characteristic brain MRI findings and a consecutive histopathological examination.
CONCLUSIONS: This is the first report of a patient with acute hyperammonemic encephalopathy together with characteristic brain MRI findings and a histopathological correlation. Although characteristic MRI findings of acute hyperammonemic encephalopathy were present, a histopathological examination revealed only hypoxic pathology without signs of metabolic encephalopathy.

The Abernethy malformation is an extremely rare congenital, extrahepatic, portosystemic shunt. There are many problems associated with this abnormal portovenous shunting and subsequent reduced hepatic portal venous flow. With the advances in non-invasive imaging technologies, these cases are diagnosed in more numbers; however, the presentation of patients is varied and the natural history is not completely known. The presenting symptom of the portosystemic shunt is mainly hyperammonemia, leading to encephalopathy. Management varies depending on the type of shunt and its clinical course; hence, the classification of the congenital portosystemic shunt is important in these patients.

In cases of acute liver failure (ALF) with hepatic coma, early liver transplantation, including ABO-incompatible (ABOi) living donor liver transplantation (LDLT), should be considered. The ABO antibody barrier can be reduced using plasma exchange (PE) and the anti-CD20 antibody rituximab. Plasma exchange is also performed for drug-induced ALF and is effective for desensitization. Rituximab treatment usually requires 14 days. There is presently no established desensitization protocol for ABOi-LDLT for ALF. Here, we report a case of drug-induced ALF with hepatic coma, which was treated with ABOi-LDLT using PE and rituximab 8 days prior to surgery. A 33-year-old female, with a history of headaches for which she was taking analgesics daily, developed drug-induced ALF with hepatic coma. Her ABOi sister desired to become a liver donor. We initiated desensitization using rituximab (500 mg) and mycophenolate mofetil (MMF, 2000 mg/day), followed by five sessions of PE. Eight days after rituximab administration, ABOi-LDLT with splenectomy was performed. Postoperatively, the patient received local infusion via portal vein for 14 days and immunosuppression with tacrolimus, methylprednisolone, and MMF. No episode of cellular or antibody-mediated rejection (AMR) was observed. The patient was discharged uneventfully 56 days after ABOi-LDLT with no problems up to 15 months after the transplant.

Implicit (i.e., unconscious) bias frequently differs from one\'s explicit or conscious convictions. As humans, we rely on information and experiences that are repeatedly reinforced until they become reflexive, shaping our perceptions of reality. Specialty bias, a form of implicit bias specific to an individual\'s medical specialty, is a form of this bias. These cognitive processes of making assumptions aid efficient decision-making and likely confers an evolutionary advantage. However, automatic thinking can contribute to stereotyping, prejudice, and discrimination at both explicit and implicit levels. Despite a person\'s explicit beliefs evolving, the lasting implicit bias significantly impacts their behavioral interactions with individuals from stereotyped groups. We present a case of an 83-year-old non-English speaking gentleman with a reported past medical history of an ischemic stroke who presented with acute encephalopathy and fever without jaundice and Aspartate transaminase/ Alanine transaminase (AST/ALT) of 64 and 34, respectively. He was initially treated for acute meningoencephalitis in the Neurologic Intensive Care Unit. With no clinical improvement in symptoms, his care was transferred to the Internal Medicine service later that week, and it was noted that he had features consistent with liver disease. Further history-taking revealed that the patient was intermittently confused with episodes of constipation. On examination, he had palmar erythema and asterixis, and additional labs showed elevated liver enzymes and ammonia levels. Computerized Tomography of the abdomen was suggestive of cirrhosis. He was treated for hepatic encephalopathy with lactulose and rifampin, with improvement in his mental status. We believe our patient\'s clinical diagnosis was compromised by incomplete information related to a language barrier, and anchoring biases prevented a thorough history taking from the patient family and later on from the patient. Physician\'s anchoring bias, a form of implicit bias, can negatively impact outcomes in patients, especially those with limited language proficiency, due to communication barriers leading to misunderstanding of the patient\'s clinical presentation and overreliance on clinical heuristics.