Lentiform fork sign: Uremia alone or multifactorial causation?

Jaryal, Ajay; Thakur, Shikha; Pathania, Joginder Singh; Vikrant, Sanjay; Kumar, Dinesh; Verma, Lokesh

doi:10.1111/hdi.12810

Cited by 4 publications

(10 citation statements)

References 8 publications

(21 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In a case series of 11 patients with chronic kidney disease it was encountered commonly in patients who had metabolic acidosis at some point [2,3]. Diabetes mellitus is reported to have some role in causation of this basal ganglia abnormality which our patient had however her blood glucose at the time of arrival was mildly deranged, the underlying mechanism has been postulated to be due to an underlying endothelial dysfunction in chronic diabetics [4][5][6].…”

Section: Discussionmentioning

confidence: 61%

“…Her lactate level was presumed secondary to seizure and hypoxemia, but it should be noted that she was taking metformin prior to admission. The pathophysiological basis for the basal ganglia abnormality is linked to vasogenic and cytotoxic edema [2]. The neuroimaging modalities CT brain and MRI brain reveal this neuroimaging abnormality and with aggressive dialysis and renal optimization these changes can be reversible [8].…”

Section: Discussionmentioning

confidence: 99%

“…Basal ganglia are metabolically highly active deep grey matter structures. There are various toxic and metabolic insults (uraemia, hypoxia, drug overdose, infections) that result in abnormalities in basal ganglia on neuroimaging [1][2][3]. The clinical features or uremic encephalopathy include tremors, chorea-athetoid movements, convulsions and coma in patients with renal diseases.…”

Section: Introductionmentioning

confidence: 99%

“…Lentiform fork sign is a neuroradiological abnormality which can be associated with uremic encephalopathy, dialysis disequilibrium syndrome and metabolic acidosis [3][4][5]. The sign is characterised by symmetrical hyperintensities in bilateral basal ganglia surrounded by hyperintense rim which delineates Lentiform nucleus [2,3].…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Lentiform fork sign in a uremic patient with a high anion gap metabolic acidosis with seizures: a case report from North West of Ireland

Mansoor

Klerk

Lineen

et al. 2020

Egypt J Neurol Psychiatry Neurosurg

View full text Add to dashboard Cite

Background Lentiform fork sign is a neuroradiological abnormality which is encountered in the clinical practice associated with uremic encephalopathy, dialysis disequilibrium syndrome and metabolic acidosis. Case presentation We describe here a case of this neuro-radiological abnormality which was encountered in a patient with uraemia and high anion gap metabolic acidosis who presented with generalised convulsion and later had some tremor in her hands. In our patient, there were few predisposing factors which might have possibly resulted in this abnormality chronic kidney disease, diabetes mellitus, and metabolic acidosis. Conclusion The Lentiform fork sign is a rare occurrence which can be related to a long list of toxic and metabolic causes but in conjunction with metabolic acidosis in chronic kidney disease patients, it can narrow down this list of alternate diagnosis.

show abstract

Section: Discussionmentioning

confidence: 61%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Lentiform fork sign in a uremic patient with a high anion gap metabolic acidosis with seizures: a case report from North West of Ireland

Mansoor

Klerk

Lineen

et al. 2020

Egypt J Neurol Psychiatry Neurosurg

View full text Add to dashboard Cite

show abstract

“…[9][10][11] Moreover, the presence of lentiform fork sign (LFS) has recently been used as an important neurological sign for the diagnosis of uremic encephalopathy, though it has been more limited to case studies. [12][13][14][15] All of these criteria can help the physician diagnose UE in the shortest time possible; but can imaging findings alone help diagnose uremic encephalopathy at early stage? Or which of these diagnostic methods (lab blood results, imaging, and clinical examination) is preferred to another?…”

mentioning

confidence: 99%

Uremic encephalopathy: A definite diagnosis by magnetic resonance imaging?

et al. 2022

View full text Add to dashboard Cite

The aim of this study was to investigate the magnetic resonance imaging (MRI) findings for the diagnose uremic encephalopathy and describe the usefulness of MRI findings in the ultimate diagnosis of uremic encephalopathy (UE). A total of 20 patients with uremic encephalopathy admitted to the hospital were evaluated in this prospective study. The clinical manifestations, laboratory and MRI imaging findings, demographic information, and clinical outcome were analyzed for each patient. We observed that the 20 prospectively reviewed patients with UE had no involvement of the basal ganglia or the lentiform fork sign (LFS). However, two-thirds of the patients had white matter involvement, and 80% of the subjects had cerebral or cortical atrophy. The arterial blood gas (ABG) analysis revealed that 50% of the patients suffered from metabolic acidosis (n=10). The results of the present study demonstrated that although the observation of Lentiform Fork Sign and Basal Ganglia involvement in MRI of UE patients is a specific finding the absence of which does not rule out UE. Thus, simultaneous examination of clinical manifestation and laboratory test analyses, along with imaging findings, should also be taken into account.

show abstract

Atypical posterior reversible encephalopathy syndrome: A lentiform fork sign following transplantation

Appiani,

Claverie,

Klein

2024

Clinical Case Reports

View full text Add to dashboard Cite

Key Clinical MessagePosterior Reversible Encephalopathy Syndrome, typically characterized by parieto‐occipital vasogenic edema, can present atypically, as a bilateral symmetrical vasogenic edema in the basal ganglia, featuring the called “lentiform fork sign.” Prompt recognition of such variations is crucial for accurate diagnosis and tailored management, highlighting the complexity of this syndrome's manifestations.AbstractPosterior Reversible Encephalopathy Syndrome (PRES) manifests as transient neurological symptoms and cerebral edema, commonly associated with immunosuppressive drugs (ISDs) in transplant recipients. ISDs can lead to endothelial dysfunction and compromise the blood–brain barrier. Typically, PRES exhibits identifiable MRI patterns, often demonstrating vasogenic edema in the bilateral parieto‐occipital white matter. Identifying unique presentations, such as the recently observed “lentiform fork sign,” commonly seen in uremic encephalopathy, emphasizes this syndrome's broad spectrum manifestations. A 19‐year‐old male, who underwent bilateral lung and liver transplantation, experienced a bilateral tonic–clonic seizure of unknown onset 47 days post‐surgery. MRI findings revealed an unconventional PRES pattern, featuring the “lentiform fork sign” as bilateral symmetrical vasogenic edema in the basal ganglia, surrounded by a hyperintense rim outlining the lentiform nucleus bilaterally. Subsequent management, including ISD modification and magnesium supplementation, resulted in clinical and neuroimaging resolution. An almost complete clinical and radiological resolution was achieved after 14 days. The occurrence of PRES in transplant recipients highlights the intricate interplay among ISDs, physiological factors, and cerebrovascular dynamics, potentially involving direct neurovascular endothelial toxicity and disruption of the blood–brain barrier. Neuroimaging plays a pivotal role in diagnosis. The distinctive “lentiform fork sign” was observed in this patient despite the absence of typical metabolic disturbances. Management strategies usually involve reducing hypertension, discontinuing ISDs, correcting electrolyte imbalances, and initiating antiseizure drugs if necessary. Identifying the presence of the “lentiform fork sign” alongside typical PRES edema in a patient lacking renal failure emphasizes that this manifestation is not solely indicative of uremic encephalopathy. Instead, it might represent the final common pathway resulting from alterations in the blood–brain barrier integrity within the deep white matter. Understanding such atypical imaging manifestations could significantly aid earlier and more precise diagnosis, influencing appropriate management decisions.

show abstract

Lentiform fork sign: Uremia alone or multifactorial causation?

Cited by 4 publications

References 8 publications

Lentiform fork sign in a uremic patient with a high anion gap metabolic acidosis with seizures: a case report from North West of Ireland

Lentiform fork sign in a uremic patient with a high anion gap metabolic acidosis with seizures: a case report from North West of Ireland

Uremic encephalopathy: A definite diagnosis by magnetic resonance imaging?

Atypical posterior reversible encephalopathy syndrome: A lentiform fork sign following transplantation

Contact Info

Product

Resources

About