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Non-Hepatic Hyperammonaemia: an Important, Potentially Reversible Cause of Encephalopathy

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Non-Hepatic Hyperammonaemia: an Important, Potentially Reversible Cause of Encephalopathy Postgrad Med J 2001;77:717–722 717 Postgrad Med J: first published as 10.1136/pmj.77.913.717 on 1 November 2001. Downloaded from CASE REPORTS Non-hepatic hyperammonaemia: an important, potentially reversible cause of encephalopathy N D Hawkes, G A O Thomas, A Jurewicz, O M Williams, C E M Hillier, I N F McQueen, G Shortland Abstract Case reports The clinical syndrome of encephalopathy CASE 1 is most often encountered in the context of A 20 year old man was admitted to a local hos- decompensated liver disease and the diag- pital with two days of inappropriate behaviour, nosis is usually clear cut. Non-hepatic clumsiness, drowsiness, memory loss, slurred causes of encephalopathy are rarer and speech, and abdominal discomfort. Since the tend to present to a wide range of medical age of 2 years he had suVered recurrent rectal specialties with variable and episodic bleeding and investigation had revealed haem- symptoms. Delay can result in the devel- orrhoids. Bleeding from his rectum had contin- opment of potentially life threatening ued over the years but had been worse recently. complications, such as seizures and coma. On examination he was confused. His Early recognition is vital. A history of Glasgow coma scale score (GCS) was 15/15. similar episodes or clinical risk factors Neurological and general examination was nor- and early assessment of blood ammonia mal, with no stigmata of chronic liver disease. levels help establish the diagnosis. In Investigations showed a leucocytosis (leuco- × 9 addition to adequate supportive care, cyte count 22 10 /l) and serum bilirubin level investigation of the underlying cause of of 32 µmol/l. Other liver function tests, haemo- the hyperammonaemia is essential and its globin, platelet count, serum electrolytes, glu- reversal, where possible, will often result cose, clotting profile, arterial blood gas analysis, in complete recovery. Detection of an and toxicology screen were normal. Cerebro- unborn error of metabolism should lead spinal fluid examination (CSF) and computed to the initiation of appropriate mainte- tomography of the brain were normal. nance therapy and genetic counselling. Shortly after lumbar puncture his conscious (Postgrad Med J 2001;77:717–722) level decreased (GCS 4/15). Plantar reflexes became extensor and he developed tonic-clonic Keywords: hyperammonaemia; encephalopathy; porto- seizures. At this point he passed melaena stool. systemic venous shunt; urea cycle defect After transfer to our hospital his GCS score http://pmj.bmj.com/ was 6/15. The pupils were dilated but reactive to light. Plantar reflexes remained extensor Hyperammonaemia is a recognised cause of with no other focal neurological deficit. Upper University Hospital of encephalopathy characterised by episodic con- gastrointestinal endoscopy was normal. Venous Wales: Department of fusion and coma. Hepatic hyperammonaemia blood ammonia was raised at 450 µmol/l (nor- Gastroenterology is usually seen in the setting of decompensated N D Hawkes mal <80 µmol/l). A diagnosis of hyperammo- liver disease when the diagnosis is reasonably G A O Thomas naemic encephalopathy was made. on October 1, 2021 by guest. Protected copyright. straightforward because of accompanying signs Because of continued heavy gastrointestinal Department of of chronic liver disease. However, there are a bleeding his encephalopathy worsened. He Surgery number of non-hepatic causes of hyperammo- required ventilatory support and his intracra- A Jurewicz naemia severe enough to cause confusion and nial pressure was found to be raised. A Department of coma. These cases are rare, have diverse mesenteric angiogram demonstrated unusual Neurology aetiologies, and as a result tend to present to portal vasculature with a large inferior me- O M Williams many diVerent specialties. In addition, they senteric vein forming a direct mesocaval shunt C E M Hillier may lack accompanying clinical signs, so the with the rectal veins and the systemic circula- I N F McQueen doctor is presented with a confused or uncon- tion (fig 1); the portal vein was absent. His Department of scious patient of unknown cause. Unless there inferior mesenteric vein pressure was raised at Paediatrics is a high index of suspicion the diagnosis may 16 mm Hg. There was no evidence of hepato- G Shortland easily be missed and the potential for reversibil- cellular failure and a liver biopsy was normal. ity and cure lost. In view of continued gastrointestinal bleeding Correspondence to: Dr N D Hawkes, Here we review non-hepatic hyperammo- and raised intracranial pressure emergency Department of naemia and describe three cases that illustrate surgery was performed and a superior meso- Gastroenterology, University important aspects of its pathophysiology and caval shunt formed reducing inferior me- Hospital of Wales, Heath Park, CardiV CF14 4XN, clinical management. In particular, these cases senteric vein pressure to 5 mm Hg. This UK highlight the diverse nature of the condition stopped the gastrointestinal bleeding. He [email protected] and the importance of establishing a diagnosis gradually regained consciousness and ammo- Submitted 21 August 2000 and treatment strategy, borne out by the fact nia levels returned to normal. He was dis- Accepted 15 February 2001 that all had a favourable outcome. charged home and remains well. www.postgradmedj.com 718 Hawkes, Thomas, Jurewicz, et al Postgrad Med J: first published as 10.1136/pmj.77.913.717 on 1 November 2001. Downloaded from Figure 1 The early phase of the mesenteric angiogram (A) shows dense contrast filling the splenic artery (SA) and a normal sized spleen. Exiting the spleen contrast flows via the splenic vein (SV) into a grossly enlarged inferior mesenteric vein (IMV). The portal vein is not seen and there is no evidence of cavernous transformation of the portal vein or collateral vessels. The filling of the inferior vena cava (IVC) on the later phase of the angiogram (B) confirms that the IMV forms a lower mesorectal shunt with the rectal veins and systemic circulation. Hyperammonaemia was thought to be due Full blood count, serum electrolytes, to the massive gastrointestinal bleed resulting glucose, liver function, chest radiography, in absorption of excess nitrogen—the ammonia abdominal ultrasound, and computed tomog- generated bypassing the liver through a distal raphy of the brain were normal. Arterial blood portosystemic shunt, allowing a significant rise gases showed mild hypoxia. CSF examination in systemic blood ammonia levels. Although a was normal, except there was a CSF protein liver biopsy was normal, the size of the nitrogen concentration of 1.67 g/l (normal <0.45 g/l). load and extent of shunting was suYcient to An electroencephalogram (EEG) showed dif- cause encephalopathy. The fact that the blood fuse non-specific abnormality. Cranial mag- ammonia level fell and the patient recovered netic resonance imaging (MRI) revealed dif- once the bleeding stopped supports this theory. fuse signal changes throughout the cerebral The development of encephalopathy during a white matter consistent with cerebral oedema. gastrointestinal bleed would be an unusual Over the next few days his conscious level complication of portal vein thrombosis—the fluctuated. He developed left sided neglect cavernous transformation and formation of with conjugate deviation of the eyes to the right http://pmj.bmj.com/ collateral vessels tend to maintain blood flow to and had several generalised tonic-clonic sei- the normal liver, preventing its occurrence. At zures, treated successfully with intravenous surgery, the portal hypertensive changes were phenytoin. His blood ammonia levels were 245 confined to the distal gastrointestinal tract with µmol/l. Urinary orotic acid and serum amino varices evident in the ileum, colon and rectum, acid levels were normal. supporting the fact that this was not a simple Hyperammonaemia was thought to be due to portal vein thrombosis. No upper gastro- slow transit constipation allowing increased intestinal varices or splenomegaly were seen, absorption of ammonia into the mesenteric on October 1, 2021 by guest. Protected copyright. suggesting that the huge anomalous inferior blood supply, suYcient to overwhelm hepatic mesenteric vein had eVectively decompressed excretory pathways. Despite lactulose and broad the proximal superior mesenteric vein and spectrum antibiotics he continued to deteriorate splenic veins preventing proximal hypertensive and required ventilation. Blood ammonia levels problems. rose to 400 µmol/l. Two days later his ureterosigmoidostomy was converted to an CASE 2 ileal conduit. He gradually improved, ammonia A 55 year old man was admitted to our hospi- levels returned to normal, and he has remained tal with two days of progressive lethargy and well. A repeat cranial MRI scan was normal. confusion. For the previous three weeks he had taken codeine phosphate for back pain and had CASE 3 developed constipation. At 6 years of age he A 10 year old boy was admitted to a local hospi- had undergone ureterosigmoidostomy for epis- tal with a two day history of headache associated padias and bladder extrophy. Subsequently he with vomiting, agitation, confusion, and slurred suVered intermittent bouts of confusion, al- speech. After a normal delivery and neonatal tered personality, and lethargy. Investigations period, he subsequently suVered a sixth nerve had found no cause. palsy (Duane’s syndrome) and a febrile seizure On examination he had a GCS of 4/15 and a during infancy. Aged 3 years he had an left sided hemiparesis. He was apyrexial, and unexplained episode of vomiting and lethargy general examination was normal apart from his lasting six days; this resolved spontaneously. At abdominal scar and epispadias. the age of 6 he was admitted to hospital with an www.postgradmedj.com Non-hepatic hyperammonaemia 719 episode of headache, vomiting, confusion, and µmol/l, respectively (normal <750 for both), Postgrad Med J: first published as 10.1136/pmj.77.913.717 on 1 November 2001. Downloaded from drowsiness associated with mild hyponatraemia serum ornithine level 15 µmol/l (normal >25), and non-specific, excess slow wave activity on and a large urinary orotic acid peak.
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