A Syndrome of Bilateral Symmetrical Basal Ganglia Lesions in Diabetic Dialysis Patients

Finelli, Pasquale F.; Singh, Joseph

doi:10.1053/j.ajkd.2013.08.030

Cited by 13 publications

(25 citation statements)

References 7 publications

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“…Typical brain MRI findings of the syndrome of acute bilateral symmetrical basal ganglia lesions in diabetic uremia consist of reversible bilateral symmetric basal ganglia lesions, with decreased signal intensity on T1-weighted images and increased signal intensity on T2-weighted images [6,9] with cytotoxic and/or vasogenic edema [[5], [6], [7], [8]]. These changes are rather specific.…”

Section: Discussionmentioning

confidence: 99%

“…Typical clinical manifestations include acute involuntary movements, dysarthria, unsteady gait or parkinsonism [[1], [2], [3], [4]]. Characteristic neuroimaging findings are reversible symmetrical lesions in bilateral basal ganglia with cytotoxic and/or vasogenic edema [[5], [6], [7], [8]]. We conducted a computerized PUBMED literature review, using the keywords “bilateral basal ganglia”, “diabetic”, and “uremia”.…”

Section: Introductionmentioning

confidence: 99%

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Unstable blood sugar levels as triggers for the syndrome of acute bilateral basal ganglia lesions in diabetic uremia: Two Taiwanese patients with unusual neuroimaging findings

Chen

Wang

2019

eNeurologicalSci

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The syndrome of acute bilateral basal ganglia lesions in diabetic uremia is uncommon and usually affects Asian patients. The underlying pathogenesis of this syndrome is not clear. We searched PUBMED using the keywords “bilateral basal ganglia”, “diabetic”, and “uremia”, and found a total of 34 cases from 1998 to 2016. In most cases, blood sugar levels were normal. Here we report two Taiwanese cases presenting with dyskinesias. In one case the syndrome was triggered by hyperglycemia, and in the other by severe hypoglycemia. Their neuroimaging findings were unusual as compared with previously reported cases, presenting as mixed hypo- and hyperintensity on T1-weighted magnetic resonance imaging. We think these new finding would shed some light on the underlying pathophysiology of this syndrome. For treatment, it is advisable to keep glucose levels as stable as possible in diabetic uremic patients to prevent this syndrome. A rapid correction of hyper- or hypoglycemia after the onset may help recovery.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Unstable blood sugar levels as triggers for the syndrome of acute bilateral basal ganglia lesions in diabetic uremia: Two Taiwanese patients with unusual neuroimaging findings

Chen

Wang

2019

eNeurologicalSci

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“…DUS can cause bilateral symmetrical basal ganglia lesions on brain CT and T2-weighted MRI 4,5 in patients with diabetic nephropathy, even if they are not on hemodialysis. To date, approximately 30 cases of DUS have been reported, many of which are Asian.…”

Section: Dus Present Casementioning

confidence: 99%

“…Ingestion of some types of mushroom, star fruit, and drugs (e.g., anti-herpes virus drugs) can cause encephalopathy in these patients [1][2][3] . In particular, diabetic dialyzed patients can present with bilateral symmetrical low densities in the basal ganglia on brain computed tomography (CT), with a bilateral symmetrical hyperintensity in the same area and a lentiform fork sign on T2-weighted MRI [4][5][6][7][8][9][10] . In addition to diabetic uremic syndrome (DUS) 4,5 , the lentiform fork sign can be observed in severe metabolic acidosis [11][12][13] , dialysis disequilibrium syndrome 14 , and metforminassociated encephalopathy (ME) 6,7 .…”

Section: Introductionmentioning

confidence: 99%

“…Herein, we present a case of a 57-year-old Japanese man in whom the lentiform fork sign was a clue for the differential diagnosis of ME or DUS. Metformin tends to increase lactate production and result in metabolic acidosis in ME 6,7,9,10 , while chronic hyperglycemia with coexistence of uremic toxins and metabolic acidosis is the main mechanism in DUS 4,5 . Which of these is the main cause in our case presenting with the lentiform fork sign is discussed below.…”

Section: Introductionmentioning

confidence: 99%

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Case Report: A case of encephalopathy presenting the lentiform fork sign on MRI in a diabetic dialysis patient - diabetic uremic syndrome or metformin-related encephalopathy?

et al. 2020

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Basal ganglia lesions showing an expansile high signal intensity on T2-weighted MRI are termed the lentiform fork sign. This specific finding is mainly observed in diabetic patients with uremic encephalopathy with metabolic acidosis, although there are also reports in patients with ketoacidosis, dialysis disequilibrium syndrome, intoxication, and following drug treatment (e.g., metformin). A 57-year-old Japanese man on chronic hemodialysis for four years because of diabetic nephropathy was admitted to our hospital for relatively rapid-onset gait disturbance, severe dysarthria, and consciousness disturbance. Brain T2-weighted MRI showed the lentiform fork sign. Hemodialysis was performed the day before admission, and laboratory tests showed mild metabolic (lactic) acidosis, but no uremia. Surprisingly, metformin, which is contraindicated for patients with end-stage kidney disease, had been prescribed for six months in his medication record, and his sluggish speaking and dysarthria appeared gradually after metformin treatment was started. Thus, the encephalopathy was considered to be related to metformin treatment. He received hemodialysis treatment for six consecutive days, and his consciousness disturbance and dysarthria improved in one week. At the eight-month follow-up, the size of the hyperintensity area on MRI had decreased, while the mild gait disturbance remained. Considering the rapid onset of gait and consciousness disturbance immediately before admission, diabetic uremic syndrome may also have occurred with metformin-related encephalopathy, and resulted in the lentiform fork sign, despite the patient showing no evidence of severe uremia on laboratory data.

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Uremic encephalopathy manifesting with a unique MRI finding (the lentiform fork sign) in an adult male: A case report

Alhusseini,

Hamsho,

Alabdullah

et al. 2023

Clinical Case Reports

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Key Clinical MessageA novel radiologic sign in patients with renal failure and uremic encephalopathy (UE) with metabolic acidosis has recently been identified as the lentiform fork sign. On magnetic resonance imaging (MRI), the “lentiform fork sign” has been described as bilateral symmetrical hyperintensities in the basal ganglia encircled by a hyperintese rim delineating the lentiform nucleus. Changes in uremic solute retention, aberrant blood–brain barrier transport, disordered vascular reactivity, altered electrolyte and acid–base balance, and altered hormone metabolism are the most likely causes of the condition. A 56‐year‐old male with end‐stage renal disease was brought to the emergency room for a progressive change in mental status and involuntary arm movements over the previous 5 days, which were accompanied by mild dyspnea. A brain MRI was performed, and it revealed hyperintensity on T2/FLAIR in the white matter surrounding the basal ganglia. the patient was treated with dialysis and improved greatly. Intensified hemodialysis and glycemic control are the cornerstones of treating diabetic uremic syndrome (DUS) with likely reversible clinical symptoms and remission of imaging abnormalities.

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A Syndrome of Bilateral Symmetrical Basal Ganglia Lesions in Diabetic Dialysis Patients

Cited by 13 publications

References 7 publications

Unstable blood sugar levels as triggers for the syndrome of acute bilateral basal ganglia lesions in diabetic uremia: Two Taiwanese patients with unusual neuroimaging findings

Unstable blood sugar levels as triggers for the syndrome of acute bilateral basal ganglia lesions in diabetic uremia: Two Taiwanese patients with unusual neuroimaging findings

Case Report: A case of encephalopathy presenting the lentiform fork sign on MRI in a diabetic dialysis patient - diabetic uremic syndrome or metformin-related encephalopathy?

Uremic encephalopathy manifesting with a unique MRI finding (the lentiform fork sign) in an adult male: A case report

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