Objective: We report two cases of movement disorders (MD) due to hyperammonemia. The first patient presented with noncirrhotic hyperammonemia after gastric bypass surgery(GBS) and the second had cirrhotic hyperammonemia mimicking MSA-C.

Background: Hyperammonemia is a recognized cause of encephalopathy, and it is commonly seen in patients with liver disease. Hyperammonemic encephalopathy following GBS is rare but highly fatal if not diagnosed in time and managed aggressively. Worldwide, there have been less than 25 reported cases of hyperammonemic encephalopathy after in the absence of cirrhosis.

Method: The first case is a man, 43 years-old, previously treated with GBS that started with major depressive mood disorder and behavior changes. In the sequence he showed cognitive decline and cerebellar signs. He scored only 19 (30) points in the minimental and suffered with daytime sleepiness. Other symptoms included dysarthria, ataxia, dysmetria, dysdiadochokinesia and tremor.The serum ammonia level was 193 micromol/L and the liver function was normal. The Brain MRI (T2/Flair weighted) demonstrated bilateral symmetric hypersignal affecting the periventricular white matter, spinal tract and cerebellar peduncles. The second patient is a 81 years-old male with late onset bilateral tremors, initially diagnosed as essential tremor. Treatment with primidone and propranolol. As the tremors worsened, cerebellar and parkinsonian signs and symptoms showed up accompanied by cognitive decline. Initial minimental was 27 the last decreased to 12 points. The Brain MRI AX Flair showed diffuse brain atrophy, with Koedan 2 and Fazekas 1 and the brain SPECT with TRODAT-1 was normal. Further imaging and laboratory tests demonstrated he had cryptogenic cirrhosis and he presented elevated serum ammonia level (137 micromol/L ).

Results: The patients were medicated with oral supplements of Ornithine aspartate 600 mg twice daily, lactulone 10 ml thrice daily and rigorous nutrition plan.They showed good response of the cerebellar and encephalopathic state.

Conclusion: The two case presented life-threatening hyperammonemic encephalopathy with cognitive and cerebellar symptoms and the oral supplements with Ornithine aspartate twice daily helped the patient. For the patient with morbid obesity, as described in literature, it is very unlikely to indicate him for reversion the original operation  given the technical implications of the surgical procedure.

References: 1) Odigwe CC, Khatiwada B, Holbrook C, et al. Noncirrhotic hyperammonemia causing relapsing altered mental status. Proc (Bayl Univ Med Cent). 2015;28(4):472-4. 2) Nagarur A, Fenves AZ. Late presentation of fatal hyperammonemic encephalopathy after Roux-en-Y gastric bypass. Proceedings (Baylor University Medical Center). 2017;30(1):41-43. 3) Bigot A, Brunault P, Lavigne C, et al. Psychiatric adult-onset of urea cycle disorders: A case-series. Mol Genet Metab Rep. 2017;12:103-109. 4) Ba F, Siddiqi ZA.Neurologic complications after bariatric surgery . Rev Neurol Dis. 2010;7(4):119-24.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/hyperammonemic-encephalopathy-as-primary-cause-of-movement-disorders-report-of-two-cases/