Portosystemic shunts: An underdiagnosed but treatable cause of neurological and psychiatric disorders

Abstract Portosystemic shunts (PSS) remain an unrecognized cause of neurological or psychiatric disorders. Here we report 5 patients with neuropsychiatric presentations of PSS. Main presentations encompassed progressive Parkinsonism, organic psychosis, recurrent coma, recurrent delusion, cognitive d...

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Bibliographic Details
Published in:Journal of the neurological sciences 2012-10, Vol.321 (1), p.58-64
Main Authors: Degos, Bertrand, Daelman, Laure, Huberfeld, Gilles, Meppiel, Elodie, Rabier, Daniel, Galanaud, Damien, Magis, Anne-Sophie, Lyon-Caen, Olivier, Samuel, Didier, Sedel, Frédéric
Format: Article
Language:English
Subjects:
Adult
Aged
Ammonia
Biological and medical sciences
Electroencephalography
Encephalopathy
Female
Humans
Hyperammonemia
Indexing in process
Magnetic Resonance Spectroscopy
Male
Medical sciences
Mental Disorders - diagnosis
Mental Disorders - etiology
Middle Aged
MRI
MRS
Nervous system (semeiology, syndromes)
Nervous system as a whole
Nervous System Diseases - diagnosis
Nervous System Diseases - etiology
Neurology
Organic psychosis
Portasystemic Shunt, Surgical - adverse effects
Portosystemic shunts
Ultrasonography
Young Adult
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Summary:Abstract Portosystemic shunts (PSS) remain an unrecognized cause of neurological or psychiatric disorders. Here we report 5 patients with neuropsychiatric presentations of PSS. Main presentations encompassed progressive Parkinsonism, organic psychosis, recurrent coma, recurrent delusion, cognitive decline and posterior cortical atrophy. None of our patients had a known history of liver disease and laboratory analyses of liver function were normal or only slightly perturbed. Only 16 similar cases of PSS revealed by neurological or psychiatric symptoms were found in the English literature. Clinical presentations were similar to our patients but asterixis, cerebellar symptoms and spastic paraparesis were noticed in some cases. EEG could be normal or could show non specific slow waves or even, rarely, triphasic slow waves. The most frequent and specific diagnostic features included hyperammonemia, abnormal brain magnetic resonance spectroscopy and visualization of the shunts by ultrasonography or abdominal imaging techniques. Therefore, in otherwise unexplained neuropsychiatric disturbances, ammonia should be routinely measured and, if elevated, a dedicated gastroenterologist or an expert radiologist should be consulted for potential PSS examination. Treatment of the shunts or of the hyperammonemia resulted in marked neurological or psychiatric improvement in all cases.
ISSN:0022-510X
1878-5883
DOI:10.1016/j.jns.2012.07.050