Lentiform Fork Sign: A Magnetic Resonance Finding in a Case of Acute Metabolic Acidosis

Grasso, Daniela; Borreggine, Carmela; Perfetto, F.; Bertozzi, Vincenzo; Trivisano, Marina; Specchio, Luigi Maria; Grilli, Gianpaolo; Macarini, Luca

doi:10.15274/nrj-2014-10041

Cited by 35 publications

(27 citation statements)

References 22 publications

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“…The LFS may also be present in metabolic acidosis from any cause, including end-stage renal disease, methanol intoxication, and the dialysis disequilibrium syndrome. [1][2][3][4][5][6] This study was undertaken to describe the MR imaging features in the brains of 10 patients with UE, with the aim of identifying common imaging features and potentially clarifying the possible pathophysiology of UE.…”

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confidence: 99%

Uremic Encephalopathy: MR Imaging Findings and Clinical Correlation

Kim

Lee

Song

2016

AJNR Am J Neuroradiol

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confidence: 99%

Uremic Encephalopathy: MR Imaging Findings and Clinical Correlation

Kim

Lee

Song

2016

AJNR Am J Neuroradiol

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“…Cortical or subcortical areas of the brain, white matter, and basal ganglia can show abnormalities. 4,5 This sign has been reported in patients with UE, but also with dialysis disequilibrium syndrome, and also has been proposed to be associated with metabolic acidosis of any cause. 1,2 Patients with diabetes may be more predisposed because of preexisting endothelial dysfunction, making them more vulnerable to uremic toxins.…”

Section: Discussionmentioning

confidence: 83%

“…Involvement of basal ganglia is more common in Asian patients with diabetes mellitus. [5][6][7] Some authors have proposed that the lentiform fork sign is an early and reliable marker of UE and have shown it to resolve after intensive hemodialysis. Wang et al have demonstrated reduced to absent glucose metabolism in basal ganglia on F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET) in patients with diabetes and uremia and basal ganglia affliction.…”

Section: Discussionmentioning

confidence: 99%

“…3 The basal ganglia are paired deep gray matter structures that are metabolically highly active and are symmetrically affected in various systemic disease states like uremia, hypoxia, drug overdose, osmotic demyelination, infections, and toxic insults such as methyl alcohol poisoning. [3][4][5] An MRI finding of bilateral symmetrical hyper intensities in the basal ganglia surrounded by a hyper-intense rim demarcating Lentiform nucleus from surrounding structures, has been described as "lentiform fork sign," the cause of which has been attributed to vasogenic edema. 4,5 This sign has been reported in patients with UE, but also with dialysis disequilibrium syndrome, and also has been proposed to be associated with metabolic acidosis of any cause.…”

Section: Discussionmentioning

confidence: 99%

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Lentiform fork sign: Uremia alone or multifactorial causation?

Jaryal

Thakur

Pathania

et al. 2019

Hemodialysis International

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Neurological complications are common in patients with acute or chronic renal failure, especially when there is marked reduction in the glomerular filtration rate (GFR). One such clinical syndrome, uremic encephalopathy (UE), occurs due to widespread dysfunction of central nervous system (CNS). It manifests with myriad clinical features and usually is suggested by bedside elicitation of asterixis (flapping tremor). Symptomatic involvement of the basal ganglia manifesting as choreoathetosis and clinical and radiological resolution with hemodialysis has been reported in the medical literature, but only rarely. The present report details such a case.

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“…Severe metabolic acidosis, with or without hypoxia and ischaemia, leads to changes in the cerebral microvascular anatomy and blood-brain barrier. These result in vasogenic oedema seen as the bright lentiform fork due to the differential metabolic vulnerability of neurons and astrocytes and may progress to cytotoxic oedema often seen as bilateral putaminal necrosis in severe cases 1. ‘Lentiform fork sign’ on MRI is a distinctive neuroradiological sign characterised by a bright hyperintense rim delineating the lentiform nucleus as a fork 2.…”

Section: Descriptionmentioning

confidence: 99%

Lentiform fork sign due to severe metabolic acidosis

et al. 2017

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DescriptionAn 11-month-old, developmentally normal, female infant presented with acute-onset fever, vomiting, shallow breathing and gradually progressive lethargy for the past 2 days. There was no history of seizures, neck stiffness, focal motor deficits, cranial nerve palsies, tone changes, rash, diarrhoea, recurrent encephalopathies, abnormal body odour, recurrent infections or hospitalisations, drug or toxin ingestion. Her past and family history was not contributory. On examination, anthropometric parameters were normal for age. She had mild pallor, angular cheilitis, wrist widening, tachypnea (respiratory rate 58/min) with acidotic breathing. She was conscious with fluctuations in alertness (Glasgow Coma Scale (GCS) E4M5V2) and irritability with reduced spontaneous activity, mild hypotonia, preserved antigravity movements, brisk muscle stretch reflexes, bilateral extensor plantar response, absence of oculomotor abnormalities, cranial nerve palsies or bulbar signs and normal fundi. Rest of the systemic examination was unremarkable. A clinical diagnosis of acute febrile encephalopathy was considered.Laboratory investigations showed haemoglobin 85 g/L, platelets 86 000 cells/µL (range 150 000-400 000), total leucocyte count 9500 cells/µL (range 4000-11 000) (neutrophils 51%, lymphocytes 41%, monocytes 7% and eosinophils 1%), serum sodium 146 meq/L, potassium 4 meq/L, urea 36 mg/dL, creatinine 0.9 mg/ dL, C reactive protein 56 mg/dL (normal below 8 mg/dL), B 12 132 pg/mL (range 211-911), homocysteine 3.86 μmol/L (range 5-15), pH 7.21, serial lactate values 4.5 and 1.4 mmol/L (normal <2), ammonia 80 μmol/L and normal anion gap. Blood glucose records were normal throughout the hospital stay. Cerebrospinal fluid analysis showed no cells, glucose 149 mg/dL and protein 152 mg/ dL. Blood, urine and cerebrospinal fluid cultures were sterile. MRI of the brain showed bilateral basal ganglia involvement with characteristic lentiform fork sign and a lactate peak on magnetic resonance spectroscopy ( figure 1A-E). Urine analysis did not reveal any reducing substances, methyl malonic acid metabolites, sulfites, glycosaminoglycans or ketones. Tandem mass spectroscopy/gas chromatography was normal. Metabolic acidosis gradually improved with supportive treatment and recovery from febrile illness, and she was discharged after 2 weeks. Follow-up evaluation after 2 months showed mild bradykinesia on neurological examination, and repeat brain MRI revealed remarkable resolution of previously reported lesions.We describe a case of acute metabolic acidosis associated with acute febrile encephalopathy, bilateral basal ganglia involvement and characteristic lentiform fork sign. The cause for metabolic acidosis was probably recurrent vomiting for the past 2 days associated with poor intake and lethargy. Underlying infection or overwhelming sepsis was also a contributory factor to the initial acidosis. Severe metabolic acidosis, with or without hypoxia and ischaemia, leads to changes in the cerebral microvascular anatomy and blood-brain b...

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Lentiform Fork Sign: A Magnetic Resonance Finding in a Case of Acute Metabolic Acidosis

Abstract: SUMMARY

Cited by 35 publications

References 22 publications

Uremic Encephalopathy: MR Imaging Findings and Clinical Correlation

Uremic Encephalopathy: MR Imaging Findings and Clinical Correlation

Lentiform fork sign: Uremia alone or multifactorial causation?

Lentiform fork sign due to severe metabolic acidosis

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