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A , The cerebellar hemispheres ( asterisk s) and dentate nuclei ( arrows ) show T2 prolongation. B , The dorsal striatum (putamen, black arrows ; caudate nucleus, white arrows ) shows bilateral hyperintense alterations.
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We present the neuroimaging and clinical findings in 2 nonalcoholic adult patients with WE as assessed by MR imaging. The first patient presented with gait ataxia and changes in consciousness. MR imaging disclosed bilateral lesions in the dorsal striatum and cerebellum. None of the regions typically affected in WE were involved. The second patient...
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Citations
... Until now, the observed MRI-pattern with exclusive symmetric basal ganglia involvement was only described in children and seemed to discriminate pediatric from adult WE [7]. In a case report, two adult patients with malnutrition in the absence of alcohol dependency also showed basal ganglia involvement, in addition to the affection of the typical regions [6,8]. The characteristic clinical picture and fast response to vitamin-B1 replacement along with the history of the patient confirmed the diagnosis of WE. ...
... Neuroimaging findings MRI showed more than 1 SD volume reduction in the thalamus, the MBs, and the vermis, as severe as in typical alcoholic KS. 43 Atrophy was also found in the basal ganglia, a brain area that can be affected in WKS. 59 However, contrary to findings in patients with alcohol use disorder, 60 there was no cortical atrophy. This underscores the importance of thiamine deficiency in the development of KS in the present patient as opposed to a direct effect of alcohol. ...
Background: Wernicke’s encephalopathy (WE) and Korsakoff syndrome (KS) are underdiagnosed. The DSM-5 has raised the diagnostic threshold by including KS in the major neurocognitive disorders, which requires that the patient needs help in everyday activities.
Methods: We report clinical, neuropsychological, and radiological findings from a patient who developed Wernicke-Korsakoff syndrome as a result of alcohol use and weight loss due to major depression. We assess the diagnosis in the context of the scientific literature on KS and according to the DSM-IV and the DSM-5.
Results: The patient developed ataxia during a period of weight loss, thus fulfilling current diagnostic criteria of WE. WE was not diagnosed, but the patient partially improved after parenteral thiamine treatment. However, memory problems became evident, and KS was considered. In neuropsychological examination, the Logical Memory test and the Word List test were abnormal, but the Verbal Pair Associates test was normal (Wechsler Memory Scale-III). There were intrusions in the memory testing. The Wisconsin Card Sorting Test was broadly impaired, but the other test of executive functions (difference between Trail Making B and Trail Making A tests) was normal. There was atrophy of the mammillary bodies, the thalamus, the cerebellum, and in the basal ganglia but not in the frontal lobes. Diffusion tensor imaging showed damage in several tracts, including the uncinate fasciculi, the cinguli, the fornix, and the corona radiata. The patient remained independent in everyday activities. The patient can be diagnosed with KS according to the DSM-IV. According to the DSM-5, the patient has major neurocognitive disorders.
Conclusions: Extensive memory testing is essential in the assessment of KS. Patients with a history of WE and typical clinical, neuropsychological, and radiological KS findings may be independent in everyday activities. Strict use of the DSM-5 may worsen the problem of Wernicke-Korsakoff syndrome underdiagnosis by excluding clear KS cases that do not have very severe functional impairment.
... For literature reviewing, we used the PubMed data base to search the term of BWernicke's encephalopathy^and the words of Bnon-alcoholic^, Bcorticalô r Bcortex^. We searched for case reports of non-alcoholic WE with cortical image abnormalities published in English languages with data reported at the individual level (Bonucchi et al. 2008;Chen et al. 2015;Cui et al. 2012;D'Aprile et al. 2000;Doss et al. 2003;Foster et al. 2005;Iannelli et al. 2010;Liu et al. 2006;Luigetti et al. 2009;Lyons et al. 2016;Mascalchi et al. 1999;Nolli et al. 2005;Ghosh et al. 2014;Pereira et al. 2011;Rufa et al. 2011;Sakurai et al. 2009;Santos Andrade et al. 2010;Sohoni 2014;Wicklund and Knopman 2013;Wu et al. 2016;Yamashita and Yamamoto 1995;Yoon et al. 2009;Zhong et al. 2005;Zuccoli et al. 2011;). One article discussing infant cases aged 2-10 months using soybean based diet was excluded (Kornreich et al. 2005). ...
In this study, we present the clinical manifestations, brain magnetic resonance imaging (MRI) and concurrent polyneuropathies in two patients with non-alcoholic Wernicke's encephalopathy (WE) after gastrojejunostomy (Billroth II) anastomosis procedures. These patients developed sub-acute onset of disorientation and disturbance of consciousness following several weeks of poor intake. Peripheral neuropathy of varying severity was noted before and after the onset of WE. Brain MRI of the patients showed cerebellar vermis and symmetric cortical abnormalities in addition to typical WE changes. Electrophysiological studies demonstrated axonal sensorimotor polyneuropathy. Prompt thiamine supplement therapy was initiated and both patients gradually recovered, however mild amnesia was still noted 6 months later. We reviewed non- alcoholic WE with atypical cortical abnormalities in English language literatures and identified 29 more cases. Eight out of 31 (25.8%) patients died during follow-up. Nine patients with gait disturbance or motor paresis had showed hyporeflexia in neurological examinations. In addition to classic triad, seizure was recorded in seven patients. Dietary deprivation is a risk factor for non-alcoholic WE among elderly patients receiving gastrointestinal surgery. The prognosis is good after thiamine supplement therapy. Recognizing the MRI features and predisposing factors in patients who have undergone gastrectomy can aid in the diagnosis and management.
... Our patient population with BBGD shares multiple neuroradiologic findings observed in Wernicke encephalopathy (WE), [8][9][10] such as involvement of the medial dorsal nucleus in the thalami (same butterfly appearance), periventricular regions of the third ventricle, brainstem, central gray matter, basal ganglia, and cerebellum. However, based on the study at hand, the main MRI difference between BBGD and WE is that in BBGD mamillary bodies are spared and that BBGD leads to a more extensive infra-and supratentorial cortical involvement compared with that observed in WE. 10 Magnetic resonance spectroscopy depicts lactate peaks and decreased N-acetylaspartate in injured areas, accounting for an early brain injury and neuronal loss; these findings are nonspecific. ...
... Wernicke ensefalopatisi (WE), tiamin (vitamin B1) eksikliği sonucu oluşan akut nörolojik bir tablodur. Genellikle alkolizm ile ilişkili olup klasik klinik triadı okülomotor anormallikler (aniden ortaya çıkan nistagmus, lateral rektus kası felci), yürüyüş ataksisi ve konfüzyon ile karakterizedir (1)(2)(3)(4)(5)(6)(7)(8)(9). Bununla birlikte, klinik triadın hastaların yaklaşık %10-20 gibi az bir kısmında görülmesi nedeniyle özellikle alkolizm öyküsü olmayan olgularda erken tanıda güçlük yaşanmaktadır (10). ...
... Bununla birlikte, klinik triadın hastaların yaklaşık %10-20 gibi az bir kısmında görülmesi nedeniyle özellikle alkolizm öyküsü olmayan olgularda erken tanıda güçlük yaşanmaktadır (10). Non-alkolik WE'nin etiyolojisinde; sık diyaliz, hiperemezis, gastrik by-pass, uzamış açlık, uzun süreli intravenöz beslenme, insan immün yetmezlik virüsü (HIV) taşıyan hasta grubu ve tiamin absorbsiyonunda bozulmaya yol açabilecek durumlar sayılabilir (5,7,8). Ciddi mortalite riski taşıması ve tiamin tedavisi ile hızlı düzelme olması nedeniyle alkolizm dışında malabsorbsiyon oluşturabilecek durumlar da ayırıcı tanıda bulundurulmalıdır (5,9). ...
... Pediatrik popülasyonda ise en sık neden malignitelerdir (11). Klasik klinik triad (1)(2)(3)(4)(5)(6)(7)(8)(9). Ancak bu bulguların tamamı hastaların sadece %10-20 oranında az bir kısmında görülmektedir (10). ...
Wernicke's encephalopathy (WE) is an acute neurologic syndrome caused by thiamine deficiency. This disease is often associated with alcoholism and the classic clinical triad is characterized by ocular abnormalities, ataxia, and confusion. However, the classic clinical triad is encountered in only 10-20% of cases. For this reason, there are difficulties in early diagnosis, especially in patients without a history of alcoholism. Due to the risk of mortality, and the rapid improvement following thiamine treatment, conditions that may lead to gastrointestinal malabsorption should be considered among reasons other than alcoholism in the differential diagnosis. In this study, we present cranial magnetic resonance imaging (MRI) findings of a patient who underwent gastric bypass surgery due to morbid obesity.
... Our patient population with BBGD shares multiple neuroradiologic findings observed in Wernicke encephalopathy (WE), [8][9][10] such as involvement of the medial dorsal nucleus in the thalami (same butterfly appearance), periventricular regions of the third ventricle, brainstem, central gray matter, basal ganglia, and cerebellum. However, based on the study at hand, the main MRI difference between BBGD and WE is that in BBGD mamillary bodies are spared and that BBGD leads to a more extensive infra-and supratentorial cortical involvement compared with that observed in WE. 10 Magnetic resonance spectroscopy depicts lactate peaks and decreased N-acetylaspartate in injured areas, accounting for an early brain injury and neuronal loss; these findings are nonspecific. ...
Objective:
To investigate the clinical, genetic, and neuroradiologic data of biotin-responsive basal ganglia disease (BBGD) and clarify the disease spectrum.
Methods:
We first investigated all patients attending our Division of Pediatric Neurology with a genetically proven diagnosis of BBGD between 2009 and 2011. All patients underwent a detailed medical history and clinical examination, extensive laboratory investigations including genetic tests, and brain MRI. Finally, we conducted a systematic review of the literature.
Results:
We enrolled 10 patients meeting the diagnostic criteria for BBGD, and analyzed the data on 14 patients from 4 previous reports. The BBGD occurred predominantly in preschool/school-aged patients in the Saudi population, but it was also observed in other ethnic groups. The typical clinical picture consisted of recurrent subacute encephalopathy leading to coma, seizures, and extrapyramidal manifestations. The brain MRI typically showed symmetric and bilateral lesions in the caudate nucleus and putamen, infra- and supratentorial brain cortex, and in the brainstem. Vasogenic edema characterized the acute crises as demonstrated by diffusion-weighted imaging/apparent diffusion coefficient MRI. Atrophy and gliosis in the affected regions were observed in patients with chronic disease. Early treatment with a combination of biotin and thiamine resulted in clinical and neuroradiologic improvement. Death and neurologic sequelae including dystonia, mental retardation, and epilepsy were observed in those who were not treated or were treated late.
Conclusion:
BBGD is an underdiagnosed pan-ethnic treatable condition. Clinicians caring for patients with unexplained encephalopathy and neuroimaging showing vasogenic edema in the bilateral putamen and caudate nuclei, infra- and supratentorial cortex, and brainstem should consider this disorder early in the hospital course because a therapeutic trial with biotin and thiamine can be lifesaving.
... Late, atrophic WE lesions also show restricted diffusion (Halavaara, Brander et al. 2003; Lapergue, Klein et al. 2006; Unlu, Cakir et al. 2006; Zuccoli, Santa Cruz et al. 2009). The most frequent conventional MRI findings in active WE are bilateral, symmetric, contrast enhancing or not, T2, late echo T2 and/or FLAIR hyperintensity of the mamillary bodies, anterior and medial nuclei of the thalamus, dorsomedial thalamus, periaqueductal grey matter periventricular grey matter, inferior and superior colliculi, caudate nuclei, midbrain and cerebellum (Schroth, Wichmann et al. 1991; Chu, Kang et al. 2002; Zhong, Jin et al. 2005; Unlu, Cakir et al. 2006; Zuccoli, Gallucci et al. 2007; Zuccoli, Cravo et al. 2011). The characteristics of the MRI lesions change along the course of the disease. ...
... The regions that frequently show contrast enhancement are the mamillary bodies, followed by the tectal plate, thalamus and periaqueductal grey matter. MRI signal changes involving the fornix, dorsal medulla oblongata, central pons, globus pallidus, putamen, frontal and/or parietal cortex, splenium, dentate nuclei, red nuclei and cranial nerve nuclei have rarely been reported (Park, Kim et al. 2001; Weidauer, Nichtweiss et al. 2003; Zuccoli, Gallucci et al. 2007; Nixon, Jordan et al. 2008; Sullivan and Pfefferbaum 2009; Zuccoli, Cravo et al. 2011). WE cortical lesions comprising of linear more or less symmetric FLAIR and/or T2 hyperintensities have also been described. ...
A 30-year-old man admitted with renal dysfunction (serum creatinine, 8.19 mg/dL) was diagnosed with immunoglobulin A nephritis through a renal biopsy. He was treated with intravenous methylprednisolone pulse therapy and urgent hemodialysis, and eventually, he underwent maintenance hemodialysis. On day 108, he developed amnesia. Magnetic resonance imaging revealed bilateral basal ganglia lesions. Wernicke encephalopathy (WE) was diagnosed based on decreased serum thiamine concentration (12.8 μg/dL; reference range, 24-66 μg/dL). Thiamine replacement therapy was initiated, but the Wernicke-Korsakoff syndrome persisted. Careful monitoring of thiamine is required in patients undergoing dialysis. In addition, patients with WE may exhibit bilateral basal ganglia lesions.
The aim of the study was to report a case of Wernicke encephalopathy (WE) due to fedratinib (Janus Kinase 2 inhibitor) treatment with atypical neuroimaging findings.
We present a detailed report of the case and literature review.
A 68-year-old woman under treatment with fedratinib (investigational JAK2 inhibitor) developed memory impairment, diplopia, and ataxia compatible with WE. Brain magnetic resonance imaging showed extensive lesions involving medial thalami, periaqueductal gray, caudate nuclei, and putamina. Thiamine supplementation provided clinical recovery and radiological improvement of the lesions described. Basal ganglia lesions have been previously described in children with this disease, but this is rarely found in adults. Clinical trials including fedratinib have been recently discontinued, and its involvement in pathogenesis of WE may be related to thiamine-transporter inhibition.
Our case represents an example of drug-related WE, with a rare radiological pattern. Precocious diagnosis and treatment are essential to prevent irreversible brain injury.
