Isolated Pulvinar/Hockey Stick Sign in Nonalcoholic Wernicke’s Encephalopathy

Khan, Faisal; Sharma, Neha; Din, Moin Ud; Bansal, Vivek

doi:10.12659/ajcr.928272

Cited by 4 publications

(12 citation statements)

References 25 publications

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“…The PS has also been described in neurological disorders of different etiologies, suggesting their sensitivity of the PN. The diseases that have reported the PS included: Tay Sachs, Krabbe disease, CNS Infections, radiation [6], variant Creutzfeldt-Jakob disease [11], anti CV2 encephalitis [11], Wernicke encephalopathy [28], Anti HU encephalitis [13], limbic encephalitis [29], neurosarcoidosis [30], and Post Influenza Encephalitis [31].…”

Section: The Pulvinar the Pulvinar Sign And Pathophisiologymentioning

confidence: 99%

Pulvinar Sign, Stroke and Their Relationship with Fabry Disease: A Systematic Review and Metanalysis

Ortíz,

Solís,

Ali

et al. 2022

Neurology International

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Background: Fabry disease (FD) is the second most common lysosomal storage disorder. This disorder affects multiple systems that include the cardiac, renal, and nervous system. The pulvinar sign (PS) is a relatively common sign seen in patients with FD. The PS is a bilateral, symmetrical pulvinar high signal relative to the signal intensity seen on unenhanced T1-weighted brain MR imaging. Methods: We conducted a systematic review with metanalysis to analyze the pool prevalence of the disorder. We used the Moose Guidelines and PRISMA Protocol for this systematic review and Robins 1 to access the BIAS of the study. To analyze the pool prevalence, we used “Open Meta-Analysis” software for analyzing the study. We used “Review Manager 5.4” to analyze the odds ratio between patients with and without the PS and patients with and without stroke among patients with FD. Results: We gather 12 studies from 2003 to 2021 for the analysis of this study. The pool prevalence of the study was 0.146 (0.076–0.217) (62/385 cases) with a 95% CI (0.0945–0.415) (p < 0.01). The prevalence was much higher in men (59 cases) than in women (3 cases). There was no relationship between the pulvinar sign and patients with stroke among patients with Fabry disease. Odds ratio 1.97 95% CI (0.35–11.21), p = 0.44; Tau2 = 0.77. There seems to be a correlation with renal failure (RF), but there were very few studies to conduct a metanalysis with RF. Conclusions: The prevalence of the PS among all studies was 23.9%; the prevalence of this sign is higher among males. We found that FD patients who had strokes did not have higher odds of presenting with the Pulvinar Sign than the FD patients who did not suffer a stroke. Patients with renal failure and FD seem to have a higher tendency to have the PS, but there were not enough studies to analyze that theory. Overall, we think the pulvinar sign has a poor prognostic value in patients with Fabry’s disease.

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Section: The Pulvinar the Pulvinar Sign And Pathophisiologymentioning

confidence: 99%

Pulvinar Sign, Stroke and Their Relationship with Fabry Disease: A Systematic Review and Metanalysis

Ortíz,

Solís,

Ali

et al. 2022

Neurology International

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“…WE, formerly known as polioencephalitis hemorrhagica superioris, is a neurological syndrome caused by thiamine deficiency [ 1 - 4 , 6 ]. The classic triad of WE includes altered mentation, gait ataxia, and ophthalmoplegia [ 1 , 2 , 7 , 8 , 10 - 14 , 17 , 18 ]. While the triad has withstood the test of time as diagnostic criteria, it is only present in 16-14% of the patients [ 2 , 10 , 17 ].…”

Section: Discussionmentioning

confidence: 99%

“…He associated these findings with a triad of gait ataxia, confusion, and ophthalmoplegia [ 1 - 3 , 6 , 8 , 9 ]. A full triad has been reported in less than 30% of the cases, and its absence does not rule out WE [ 1 , 2 , 8 - 10 ]. Secondary symptoms include vestibular dysfunction, hypothermia, and peripheral neuropathy [ 1 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

“Bariatric Beriberi”: A Rare Case of Wernicke Encephalopathy Two Weeks After Laparoscopic Sleeve Gastrectomy

Bathobakae¹,

Ozgur²,

Lombardo³

et al. 2023

Cureus

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Wernicke encephalopathy (WE) is an acute neurological syndrome caused by thiamine (vitamin B1) deficiency. This disorder manifests as a triad of gait ataxia, confusion, and vision abnormalities. The absence of a full triad does not rule out WE. Because of its vague presentation, WE is commonly missed in patients with no history of alcohol abuse. Other risk factors for WE include bariatric surgery, hemodialysis, hyperemesis gravidarum, and malabsorption syndromes. WE is a clinical diagnosis that can be confirmed with an MRI of the brain as hyperintensities in the mammillary bodies, periaqueductal area, thalami, and hippocampus. If suspected in a patient, WE must be immediately treated with intravenous thiamine to prevent evolution into Korsakoff syndrome, coma, or death. Currently, there is no consensus in the medical community as to how much thiamine must be given and for how long. Therefore, there is a need for more research in the diagnosis and management of WE after bariatric surgery. Herein, we report a rare case of a 23-year-old female with a history of morbid obesity who developed WE two weeks after a laparoscopic sleeve gastrectomy.

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“…Sensory ataxia can result from pathologies along the neuraxis at multiple levels. The thalamus is vulnerable to insults from multiple etiologies and, being the main sensory relay station, this can cause severe sensory gait ataxia [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Our patient’s brain MRI was reviewed by 2 independent American board-certified neuroradiologists, who raised the possibility of Wernicke’s encephalopathy as the primary diagnosis. Our group has previously reported isolated “pulvinar/ hockey stick sign” in patients with non-alcoholic Wernicke’s encephalopathy [ 8 ]. Our patient did not respond to thiamine supplementation.…”

Section: Discussionmentioning

confidence: 99%

A Missed Case of Area Postrema Syndrome Presenting with Neuromyelitis Optica Spectrum Disorder

et al. 2021

Self Cite

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Patient: Female, 33-year-old Final Diagnosis: Neuromyelitis optica Symptoms: Altered mental status • ataxia • fever • hiccups • hypersomnolence • nausea • vomiting Medication: — Clinical Procedure: Aquaporin-4 antibody serological testing • cerebrospinal fluid analysis • electroencephalogram Specialty: Gastroenterology and Hepatology • Neurology • Radiology Objective: Rare disease Background: Neuromyelitis optica spectrum disorder (NMOSD), which is also known as Devic disease, is a chronic disorder of the brain and spinal cord that includes inflammation of the optic nerve and spinal cord. Area postrema syndrome (APS) is due to involvement of the bulbar emetic reflex center, and has previously been described in NMOSD. Patients with APS may present with nausea, vomiting, or hiccups. This report is of a 33-year-old Asian American woman with history of APS who presented with NMOSD. Case Report: A 33-year-old Southeast Asian woman, 2 months postpartum, presented with fever, hypersomnolence, altered mental status, and difficulty ambulating. Neurological examination revealed a lethargic woman with poor attention span, broad-based gait ataxia, and positive Romberg’s sign. Laboratory work-up showed sodium 123 milliequivalent/L (mEq/L). Brain magnetic resonance imaging (MRI) with contrast revealed bilateral, non-enhancing, patchy fluid-attenuated inversion recovery (FLAIR) hyperintensities in the anteroinferomedial thalamus extending to the mammillary bodies. Additional history revealed hospitalization for intractable nausea, vomiting, and hiccups 2 years ago. NMOSD was confirmed with positive AQP-4 antibody, prompting treatment with intravenous (i.v.) methylprednisolone, followed by plasmapheresis. Repeat brain MRI showed mild improvement of bilateral thalamic FLAIR hyperintensities and no clinical recurrence was reported with Rituximab treatment. Conclusions: This case highlights the importance of the diagnostic diligence required for NMOSD diagnosis. Multiple etiologies can mimic the clinical presentation of acute diencephalic syndrome; thus, a broad differential needs to be considered. This report presents the diagnostic work-up and management of a patient with a complex neurological condition that was diagnosed as NMOSD.

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Isolated Pulvinar/Hockey Stick Sign in Nonalcoholic Wernicke’s Encephalopathy

Cited by 4 publications

References 25 publications

Pulvinar Sign, Stroke and Their Relationship with Fabry Disease: A Systematic Review and Metanalysis

Pulvinar Sign, Stroke and Their Relationship with Fabry Disease: A Systematic Review and Metanalysis

“Bariatric Beriberi”: A Rare Case of Wernicke Encephalopathy Two Weeks After Laparoscopic Sleeve Gastrectomy

A Missed Case of Area Postrema Syndrome Presenting with Neuromyelitis Optica Spectrum Disorder

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