Clinical Neuroscience

[Reversible hepatocerebral degeneration-like syndrome due to portovenous shunts]


NOVEMBER 30, 2013

Clinical Neuroscience - 2013;66(11-12)

[Ataxia and tremor are rare manifestations of hepatocerebral degeneration due to portovenous shunts. Ammonia is a neurotoxin that plays a significant role in the pathogenesis of hepatic encephalopathy. A 58-year old male patient was assessed with the complaints of gait disturbance, hand tremor, and impairment of speech. His neurological examination revealed dysarthric speech and ataxic gait. Bilateral kinetic tremor was noted, and deep tendon reflexes of the patient were hyperactive. Serum ammonia level was found to be 156.9 μg/dL. Cranial magnetic resonance (MR) imaging revealed increased signal intensity in bilateral globus pallidus on T1-weighted axial sections, and bilateral prominent hyperintense lesions in the middle cerebellar peduncles on T2-weighted axial sections. On his abdominal MR portography, multiple portohepatic venous collaterals were noted in the right and left lobes of liver parenchyma in 2D FIESTA axial MR sections. To our knowledge, we reported the first case of acquired hepatocerebral degeneration presenting with cerebral symptoms without any hepatic findings in which clinical improvement was noted, and hyperammonemia disappeared following medical treatment.]



Further articles in this publication

Clinical Neuroscience

[The fate of tyrosinaemic Hungarian patients before the NTBC aera]


[Before the introduction of the NTBC treatment (Orfadine) from two tyrosinemic Hungarian families 1-3 tyrosinemic homozygous male patients died of hepatocellular carcinoma and one patient of hepatocellular carcinoma combined with clear cell renal adenocarcinoma. From the third tyrosinemic family one homozygous girl patient has been treated with NTBC (Orfadine), IMTV-AM, she is symptom-free. Her molecular genetic mutations analysis in the FAH gene detected a common intronel mutation, affecting splicing and of predicted severe effect, IVS6-1 g > t/IVS6-1 g > t with systemic name c.456-1 g > t/c.456-1 g > t (Prof. Magdalena Ugarte).]

Clinical Neuroscience

[Treatment possibilities in advanced Parkinson’s disease]

TAKÁTS Annamária, NAGY Helga, RADICS Péter, TÓTH Adrián, GERTRÚD Tamás

[In the course of Parkinson’s disease, advanced and late stages can be distinguished. In the advanced stage, levodopa has good effect on motor symptoms, but patient care is often hindered by levodopa-induced complications such as motor fluctuation and dyskinesias. In the late stage levodopa response becomes poor, falls, dementia and psychotic symptoms appear and patients often need hospitalization. In the advanced stage, the quality of life may be improved better by device-aided therapy than by best oral medical treatment. The alternatives are apomorhin pump, levodopa carbidopa intestinal gel with pump and deep brain stimulation. The therapy plan should be based on the principle: “the right treatment, to the right patient, in the right time”.]

Clinical Neuroscience

[In memoriam Katalin Háberland]


Clinical Neuroscience

[Psychosis as a process - New implications of staging models of schizophrenia]


[The article discusses contributing factors in the pathogenesis of schizophrenia. In the last fifteen years, the emphasis has shifted from curative to prodromal and premorbid characteristics of later schizophrenia patients. Nevertheless, most studies are limited to the area of early detection and intervention of schizophrenia with much fewer focusing on actual prevention. A more general preventive approach not limited to psychotic condition is clearly underestimated. Following a review of current literature on prodromal approaches and identified premorbid markers of schizophrenia, the article outlines a possible trajectory of later psychotic condition with detectable, distinct stages from birth on. Based on this extended staging model involving neurotoxic impact and early prefrontal-limbic dysfunction, it argues for a refined, phase-specific treatment protocol including preventive interventions. Accepting a model of schizophrenia as an illness with detectable, phase-specific signs and symptoms from infancy on leads to the need to implement preventive interventions. Through this approach, we could, in the optimal case, be able to identify early signs of neuromotoric and cognitive dysfunction not specific for psychosis. Furthermore, it would be useful to lay greater emphasis on the detection of these early signs in the training of health care professionals. This approach calls for a close cooperation between psychologists, psychiatrists, neuropsychologists and special education experts and a change in the way we view psychotic illness.]

Clinical Neuroscience

[The Wall Steet Journal’s report about the study of neurologists and psychologists from Szeged]


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