Objective: To report an example of the rare association between acquired hepatocerebral degeneration (AHD) and ocular motor apraxia (OA), and discuss its relevance for topical diagnosis in Neurology.

Background: OA is an inability to initiate voluntary saccades in a head-fixed position, with preserved reflex saccades (initiated by the vestibulo-ocular reflex).[1] AHD is a polysymptomatic neurobehavioral syndrome occurring in the setting of advanced liver disease.[2] The most common phenotype of AHD is atypical parkinsonism and cognitive involvement is common.[2] Onset is generally subacute over several weeks to months and response to levodopa is variable.[2]

Method: A 66 year-old male presented with complaints of frequent falls over the prior 6 months. His wife reported mood fluctuations, confusion and difficulty remembering familiar names. He had been diagnosed with alcoholic cirrhosis for 12 years, Child-Pugh B, and become progressively dependent on activities of daily living.

On examination he showed cognitive impairment with bradyphreny and executive dysfunction, dysarthria and bilateral symmetrical parkinsonism. There was bilateral postural tremor (asterixis), bilateral dysmetria and ataxic gait. He was unable to elicit voluntary pursuit movements and saccades in all directions in a stationary head position, but had the ability to trigger conjugated eye movement by imagination or initiation of head movement, which was compatible with OA.

Results: The patient exhibited bilateral pallidal T1-hyperintensities on MRI, reflecting pallidal manganese deposition, as well as bilateral parietal hypersignal on T2 and bilateral restriction at the pre-frontal cortex; serum manganese levels were elevated. He was started on zinc and rifaximin, with poor clinical response. He was also started on propranolol 10mg orally with good improvement of tremor, but maintained atypical parkinsonism with gait instability and OA.

Conclusion: OA points to a dysfunction in the area of the frontal eye fields (voluntary saccade initiation); additional dysfunction in the parietal eye fields may eventually impair initiation of reflexive saccades.[3] That cortical predominance of neurodegeneration was demonstrated in our patient´s MRI. This case was semiologically very challenging since OA was present in absence of limb or orobuccal apraxia (more frequently observed manifestations of apraxia). We hope to demonstrate the relevance of this rare and scarcely reported feature of AHD.

References: 1. Pearls & Oy-sters: Ocular motor apraxia as essential differential diagnosis to supranuclear gaze palsy. Eyes up. Schweyer K, Busche MA, Hammes J, Zwergal A, Buhmann C, Eimeren T and Hoglinger GU. Neurology. 2018;90:482-5.
2. Mulroy E, Baschieri F, Magrinelli F, Latorre A, Cortelli P and Bhatia KP. Movement Disorders and Liver Disease. Movement Disorders – Clinical Practice. 2021; 8(6):828842.
3. Strupp M, Kremmyda O, Adamczyk C, et al. Central ocular motor disorders, including gaze palsy and nystagmus. J Neurol 2014;261(2 suppl):S542–S558.

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