A 58-year-old man with arterial hypertension and poorly controlled type 2 diabetes using glyburide/metformin presented a history of sudden slurred speech and gait disturbance eight days before clinical evaluation. Neurological examination disclosed dysarthria, postural instability and bradykinesia. Laboratory investigations demonstrated low glucose (24 mg/dL), elevated urea nitrogen (168 mg/dL) and creatinine (8 mg/dL) levels. Cranial computerized tomography revealed lentiform “fork sign” (Figures 1A and 1C).
Axial and coronal cranial computerized tomography (A/C) - decreased density in bilateral lentiform nuclei surrounded by a more hypodense rim delineating them - fork-like structure. Cranial computerized tomography (B/D) - regression of lentiform nucleus lesions after three months.
Acute bilateral basal ganglia lesions syndrome is a rare complication of uremia, occurring typically in diabetic patients and characterized by acute parkinsonism and/or involuntary movements11. Juryńczyk M, Rożniecki J, Zaleski K, Selmaj K. Hypoglicemia as a trigger for the syndrome of acute bilateral basal ganglia lesion in uremia. J Neurol Sci. 2010 Oct; 297(1-2):74-5. https://doi.org/10.1016/j.jns.2010.07.016
https://doi.org/https://doi.org/10.1016/...
. The pathogenesis is unclear and potential causes include metabolic acidosis11. Juryńczyk M, Rożniecki J, Zaleski K, Selmaj K. Hypoglicemia as a trigger for the syndrome of acute bilateral basal ganglia lesion in uremia. J Neurol Sci. 2010 Oct; 297(1-2):74-5. https://doi.org/10.1016/j.jns.2010.07.016
https://doi.org/https://doi.org/10.1016/...
,22. Fabiani G, Teive HA, Munhoz RP. Lentiform fork sign and fluctuating, reversible parkinsonism in a patient with uremic encephalopathy. Mov Disord 2013 Jul; 28(8):1053. https://doi.org/10.1002/mds.25503
https://doi.org/https://doi.org/10.1002/...
,33. Moreira RF, Barsottini OG, Pedroso JL. Lentiform “fork sign” and parkinsonism after acute myocardial infarction and cardiac failure. Mov Disord Clin Pract 2017 Feb; 4(4):646. https://doi.org/10.1002/mdc3.12482
https://doi.org/https://doi.org/10.1002/...
, uremic toxins11. Juryńczyk M, Rożniecki J, Zaleski K, Selmaj K. Hypoglicemia as a trigger for the syndrome of acute bilateral basal ganglia lesion in uremia. J Neurol Sci. 2010 Oct; 297(1-2):74-5. https://doi.org/10.1016/j.jns.2010.07.016
https://doi.org/https://doi.org/10.1016/...
,33. Moreira RF, Barsottini OG, Pedroso JL. Lentiform “fork sign” and parkinsonism after acute myocardial infarction and cardiac failure. Mov Disord Clin Pract 2017 Feb; 4(4):646. https://doi.org/10.1002/mdc3.12482
https://doi.org/https://doi.org/10.1002/...
, hemodynamic changes (hypotension/heart failure)33. Moreira RF, Barsottini OG, Pedroso JL. Lentiform “fork sign” and parkinsonism after acute myocardial infarction and cardiac failure. Mov Disord Clin Pract 2017 Feb; 4(4):646. https://doi.org/10.1002/mdc3.12482
https://doi.org/https://doi.org/10.1002/...
and diabetes11. Juryńczyk M, Rożniecki J, Zaleski K, Selmaj K. Hypoglicemia as a trigger for the syndrome of acute bilateral basal ganglia lesion in uremia. J Neurol Sci. 2010 Oct; 297(1-2):74-5. https://doi.org/10.1016/j.jns.2010.07.016
https://doi.org/https://doi.org/10.1016/...
. In the case reported, hypoglycemia may be implicated as a triggering factor. The patient’s symptoms improved after hemodialysis.
References
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1Juryńczyk M, Rożniecki J, Zaleski K, Selmaj K. Hypoglicemia as a trigger for the syndrome of acute bilateral basal ganglia lesion in uremia. J Neurol Sci. 2010 Oct; 297(1-2):74-5. https://doi.org/10.1016/j.jns.2010.07.016
» https://doi.org/https://doi.org/10.1016/j.jns.2010.07.016 -
2Fabiani G, Teive HA, Munhoz RP. Lentiform fork sign and fluctuating, reversible parkinsonism in a patient with uremic encephalopathy. Mov Disord 2013 Jul; 28(8):1053. https://doi.org/10.1002/mds.25503
» https://doi.org/https://doi.org/10.1002/mds.25503 -
3Moreira RF, Barsottini OG, Pedroso JL. Lentiform “fork sign” and parkinsonism after acute myocardial infarction and cardiac failure. Mov Disord Clin Pract 2017 Feb; 4(4):646. https://doi.org/10.1002/mdc3.12482
» https://doi.org/https://doi.org/10.1002/mdc3.12482
Publication Dates
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Publication in this collection
25 Nov 2020 -
Date of issue
Dec 2020
History
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Reviewed
05 Apr 2020 -
Received
13 June 2020 -
Accepted
16 June 2020