We present the case of a 54-year-old male, without any significant medical history, who insidiously developed speech disturbances and walking difficulties, accompanied by backward falls. The symptoms progressively worsened over time. The patient was initially diagnosed with Parkinson’s disease; however, he failed to respond to standard therapy with Levodopa. He came to our attention for worsening postural instability and binocular diplopia. A neurological exam was highly suggestive of a Parkinson-plus disease, most likely progressive supranuclear gaze palsy. Brain MRI was performed and revealed moderate midbrain atrophy with the characteristic “hummingbird” and “Mickey mouse” signs. An increased MR parkinsonism index was also noted. Based on all clinical and paraclinical data, a diagnosis of probable progressive supranuclear palsy was established. We review the main imaging features of this disease and their current role in diagnosis.
Background. It is known that alterations of the sleep-wake cycle are present from the early stages of hepatic encephalopathy (starting with grade I on the West Haven criteria). However, minimal hepatic encephalopathy (which is not included in the West Haven criteria) defines the earliest form of HE, with no obvious clinical manifestations, but with subtle alterations in cognitive domains such as attention, visuo-spatial perception, psychomotor speed and response inhibition. Aim. To determine if liver cirrhosis in otherwise asymptomatic individuals (without clinically manifest hepatic encephalopathy) is associated with an increased risk of altered sleep rhythm or excessive daytime sleepiness. Additionally, to determine if this risk is correlated with liver disease severity or other clinical and biological parameters Material and methods. Cross-sectional study involving 25 adult patients with liver cirrhosis. Patients were evaluated through a standard neurological examination and with the Epworth Sleepiness Scale. Liver disease severity was measured using the MELD and Child-Pugh scores. Blood ammonia levels were also measured. Results. Mean age of the patients was 50±12 years-old. There was a predominance of males (68%, n=17). Mean MELD score was 17±7 points. Mean Child-Pugh score was 8±2 points. Mean blood ammonia level was 34±17 μg/dL. Subjective daytime somnolence was reported by 24% of patients (n=6). Mean ESS score was 6±5 points. No correlation was noted between the ESS scores and liver disease severity as measured by the Child-Pugh and MELD scores. Only three patients (12%) scored over 10 points on the ESS (none of them obese) and were consequently diagnosed with excessive daytime sleepiness (EDS). This proportion falls into the accepted prevalence of EDS in the general population. Conclusions. In our study, liver cirrhosis was not associated with a higher prevalence of excessive daytime sleepiness than in the general population.
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