Alcoholic cerebellar degeneration
Alcoholic cerebellar degeneration is a common type of acquired cerebellar ataxia characterized by chronic vermian atrophy 1. It is a sequela of chronic alcohol abuse or malnutrition and has also been described in the literature as alcohol-related cerebellar degeneration, alcohol-induced cerebellar degeneration and nutritional cerebellar degeneration 1,2.
The prevalence is estimated at 12 – 27% from autopsies 2.
Risk factors are those conditions predisposing to malnutrition, including chronic alcohol abuse 2. Regarding alcohol use, the duration of excessive use is likely the main factor 3-5.
- coarse tremor
- truncal instability
- lower limb ataxia
- upper limb ataxia (in severe cases)
The exact pathogenesis is yet to be elucidated but it is commonly thought to arise from either alcohol neurotoxicity, nutritional deficiency (notably thiamine) or a combination of both 1,6.
Despite its current name, alcohol may not necessarily be a prerequisite as similar clinicopathological findings have been described in durations of abstinence or in malnourished non-alcoholics 2,6. Hence it is no wonder that alternative terms have been adopted in the literature.
Histologically, there is Purkinje cell loss in the cerebellar cortex accompanied by Bergmann gliosis, and a variable loss of the molecular layer and granule cells 7. Associated neuronal loss in the dorsal inferior olivary nuclei may also be present. The underlying cerebellar white matter is otherwise preserved 7.
Findings are similar to those in MRI.
Abnormalities are best demonstrated on sagittal views of T1-weighted images 2. Midline cerebellar structures are primarily involved.
Early features consist of cerebellar volume loss localized to the anterior superior vermis (lingula, central lobule, culmen, declive) and associated widening of inter-folial sulci 2,7,8. Subsequent progression to involve the posterior inferior vermis and anterior lobes of the cerebellum can occur 8.
These features may occur in isolation or in the presence of other radiographic abnormalities associated with chronic alcohol use, such as those in Wernicke encephalopathy 7. Correlation of radiographic findings with the clinical presentation is crucial as vermian atrophy can be present in asymptomatic individuals 2.
FDG studies may exhibit hypometabolism in similarly affected areas of the cerebellum 9.
Treatment and prognosis
Treatment is targeted towards abstinence, nutritional supplementation and gait optimization 2.
Improvement varies with the degree of abstinence and dietary repletion, although gait ataxia can be persistent in severe cases 2.
- 1. Priya D. Shanmugarajah, Nigel Hoggard, Stuart Currie, Daniel P. Aeschlimann, Pascale C. Aeschlimann, Dermot C. Gleeson, Mohammed Karajeh, Nicola Woodroofe, Richard A. Grünewald, Marios Hadjivassiliou. Alcohol-related cerebellar degeneration: not all down to toxicity?. (2016) Cerebellum & Ataxias. 3 (1): 1. doi:10.1186/s40673-016-0055-1 - Pubmed
- 2. Laureno R. Nutritional cerebellar degeneration, with comments on its relationship to Wernicke disease and alcoholism. (2012) Handbook of clinical neurology. 103: 175-87. doi:10.1016/B978-0-444-51892-7.00010-3 - Pubmed
- 3. Oscar H. Del Brutto, Robertino M. Mera, Nathan R. King, Mauricio Zambrano, Lauren J. Sullivan. Years of Drinking but Not the Amount of Alcohol Intake Contribute to the Association Between Alcoholic Cerebellar Degeneration and Worse Cognitive Performance. A Population-Based Study. (2017) The Cerebellum. 16 (2): 612. doi:10.1007/s12311-016-0824-7 - Pubmed
- 4. Alcoholic Cerebellar Degeneration Is Not a Dose‐Dependent Phenomenon. (1987) Alcoholism: Clinical and Experimental Research. 11 (4): 372. doi:10.1111/j.1530-0277.1987.tb01327.x - Pubmed
- 5. Del Brutto OH, Mera RM, Sullivan LJ, Zambrano M, King NR. Population-based study of alcoholic cerebellar degeneration: The Atahualpa Project. (2016) Journal of the neurological sciences. 367: 356-60. doi:10.1016/j.jns.2016.06.051 - Pubmed
- 6. Matthias Maschke, J. Weber, U. Bonnet, A. Dimitrova, J. Bohrenkämper, S. Sturm, B. W. Müller, M. Gastpar, H.-Ch. Diener, M. Forsting, D. Timmann. Vermal atrophy of alcoholics correlate with serum thiamine levels but not with dentate iron concentrations as estimated by MRI. (2005) Journal of Neurology. 252 (6): 704. doi:10.1007/s00415-005-0722-2 - Pubmed
- 7. Francoise Gray, Charles Duyckaerts, Umberto De Girolami. Escourolle & Poirier's Manual of Basic Neuropathology. (2013) ISBN: 9780199330485
- 8. Lee JH, Heo SH, Chang DI. Early-stage Alcoholic Cerebellar Degeneration: Diagnostic Imaging Clues. (2015) Journal of Korean medical science. 30 (11): 1539. doi:10.3346/jkms.2015.30.11.1539 - Pubmed
- 9. Sid Gilman, Kenneth Adams, Robert A. Koeppe, Stanley Berent, Karen J. Kluin, Jack G. Modell, Phillip Kroll, James A. Brunberg. Cerebellar and frontal hypometabolism in alcoholic cerebellar degeneration studied with positron emission tomography. (1990) Annals of Neurology. 28 (6): 775. doi:10.1002/ana.410280608 - Pubmed