Abstract
Acquired hepatocerebral degeneration (AHD) is a neurological disorder characterised by parkinsonism and less frequently by ataxia or pyramidal signs [1, 2]. Although some characteristics of AHD have not yet been fully defined and diagnosis-criteria do not exist, it has been reported that: (1) a variety of chronic neurological and cognitive abnormalities are associated with chronic liver failure, which are clinically distinct from the more acute and transient episodes of hepatic encephalopathy (HE); (2) in contrast to Parkinson’s disease (PD), AHD-related parkinsonism is symmetrical at onset; progresses more rapidly than PD, peak severity occurring within 1 year of symptoms onset; it is associated with early postural instability and with additional neurological signs such as chorea, ataxia and oro-buccal dyskinesia; is far less responsive to levodopa therapy; (3) heavy metals, particularly manganese have been implicated in the pathogenesis of AHD; (4) increased serum manganese concentration, and such neuro-radiological findings as basal ganglia hyperintensity on T1-weighted sequences at brain magnetic resonance imaging (MRI) are corroborative for the diagnosis of AHD. Here, we report a case of AHD with atypical MRI findings. A 63-year-old woman was admitted to our department because of a 1-year history of global slowness of movements and shuffling gait accompanied by postural instability and tremor of the upper limbs. Although onset was insidious, peak severity occurred within 7 months of onset. Neurological examination showed a severe akineticrigid symmetric syndrome with balance impairment; rest, postural and action tremor of upper limbs; slurred speech; slowness of ocular saccadic movements and up-gaze limitation. There were no prominent psychiatric symptoms, except for mild depression. The patient’s medical history was notable only for cryptogenic cirrhosis, with a ChildPugh score of 6 (Class A), but there were no fluctuations of cognitive performance, which contrasts with diagnosis of HE. The patient had taken no drugs potentially responsible for drug-induced parkinsonism. L-Dopa administered at a dose of up to 800 mg/day was without benefit. Serum manganese concentration was increased (1.2 ng/ml; normal range: 0.4–0.85 ng/ml), whereas serum ceruloplasmin, cupremia, and cupruria were within reference values. Brain MRI at 1.5 T showed symmetric hyperintensities of the basal ganglia, especially putamina and caudate nuclei, only detectable on protondensity weighted sequences (PDW) (Fig. 1a, b). [I]-FPCIT SPECT excluded dopamine transporter deficiency. Neurological evaluation 8 months later showed no remarkable change in the patient’s clinical status. The radiological follow-up revealed the persistence of the PDW abnormalities (Fig. 1c). Moreover, brain MRI showed an increased T1 signal in the bilateral pallidum (Fig. 1d), R. Erro C. Vitale M. Picillo Department of Neurological Sciences, University of Naples Federico II, Naples, Italy
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