Nonalcoholic Fatty Liver Disease in Patients with Inherited and Sporadic Motor Neuron Degeneration

Brian Johnson, Angela Kokkinis, Neville Gai, Ejaz A Shamim, Craig Blackstone, Kenneth H Fischbeck, Christopher Grunseich, Brian Johnson, Angela Kokkinis, Neville Gai, Ejaz A Shamim, Craig Blackstone, Kenneth H Fischbeck, Christopher Grunseich

Abstract

We describe evidence of fatty liver disease in patients with forms of motor neuron degeneration with both genetic and sporadic etiology compared to controls. A group of 13 patients with motor neuron disease underwent liver imaging and laboratory analysis. The cohort included five patients with hereditary spastic paraplegia, four with sporadic amyotrophic lateral sclerosis (ALS), three with familial ALS, and one with primary lateral sclerosis. A genetic mutation was reported in nine of the thirteen motor neuron disease (MND) patients. Fatty liver disease was detected in 10 of 13 (77%) MND patients via magnetic resonance spectroscopy, with an average dome intrahepatic triacylglycerol content of 17% (range 2-63%, reference ≤5.5%). Liver ultrasound demonstrated evidence of fatty liver disease in 6 of the 13 (46%) patients, and serum liver function testing revealed significantly elevated alanine aminotransferase levels in MND patients compared to age-matched controls. Fatty liver disease may represent a non-neuronal clinical component of various forms of MND.

Trial registration: ClinicalTrials.gov NCT02124057.

Keywords: amyotrophic lateral sclerosis; fatty liver; motor neuron disease; steatosis.

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
(A) 1H magnetic resonance spectroscopic imaging showed that the motor neuron disease group had a significantly higher intrahepatic triacylglycerol (IHTG) content in the dome of the liver compared to controls (** p = 0.0086); (B) increased echogenicity on liver ultrasound showing steatosis in patients with amyotrophic lateral sclerosis (ALS), juvenile ALS (JALS) with mutation in senataxin, and hereditary spastic paraplegia (HSP). Two patients from each category are shown; (C) voxel placement (red square) in the liver dome of a patient with HSP and spectroscopy trace showing the peaks for water (left asterisk) and triacylglycerol (right asterisk).
Figure 2
Figure 2
There was a significant correlation between (A) the liver dome intrahepatic triacylglycerol (IHTG) content and the serum γ-glutamyl transferase (r = 0.58, p = 0.0063) and (B) serum alanine aminotransferase and serum γ-glutamyl transferase (r = 0.41, p = 0.018) in the motor neuron disease group.

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Source: PubMed

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