[Sporadic Creutzfeldt-Jakob Disease With Slow Progression:Report of One Case].

Sporadic Creutzfeldt-Jakob disease(sCJD)is a prion-caused degenerative disease of the central nervous system,with the typical clinical manifestation of rapidly progressive dementia.The course of disease is less than 1 year in most patients and more than 2 years in only 2% to 3% patients.We reported a case of sCJD with expressive language disorder and slow progression in this paper.By summarizing the clinical manifestations and the electroencephalograhpy,MRI,and pathological features,we aimed to enrich the knowledge about the sCJD with slow progression.

A distinct clinicopathological variant of focal cortical dysplasia IIId characterized by loss of layer 4 in the occipital lobe in 12 children with remote hypoxic-ischemic injury.

OBJECTIVE: In 2011, the International League Against Epilepsy (ILAE) proposed a consensus classification system of focal cortical dysplasia (FCD) to distinguish clinicopathological subtypes, for example, "isolated" FCD type Ia-c and IIa-b, versus "associated" FCD type IIIa-d. The histopathological differentiation of FCD type I and III variants remains, however, a challenging issue in everyday practice. We present a unique histopathological pattern in patients with difficult-to-diagnose FCD, which highlights this dilemma, but also helps to refine the current ILAE classification scheme of FCD. METHODS: We present a retrospective series of 11 male and one female patient with early onset pharmacoresistant epilepsy of the posterior quadrant (mean age at seizure onset = 4.6 years). All surgical specimens were reviewed. Clinical histories were retrieved and extracted from archival patient files. RESULTS: Microscopic inspection revealed abnormalities in cortical architecture with complete loss of layer 4 in all surgical samples of the occipital lobe, as confirmed by semiquantitative measurements (p < 0.01). Clinical history reported early transient hypoxic condition in nine patients (75%). Magnetic resonance imaging (MRI) revealed abnormal signals in the occipital lobe in all patients, and signal changes suggestive of subcortical encephalomalacia were found in seven patients. Surgical treatment achieved favorable seizure control (Engel class I and II) in seven patients with an available follow-up period of 6.1 years. SIGNIFICANCE: Prominent disorganization of cortical layering and lack of any other microscopically visible principle lesion in the surgical specimen would result in this neuropathological pattern hitherto being classified as FCD ILAE type Ib. However, perinatal hypoxia with distinctive MRI changes suggested primarily a hypoxemic lesion and acquired pathomechanism of neuronal cell loss in the occipital lobe of our patient series. We propose, therefore, classifying this distinctive clinicopathological pattern as a separate variant of FCD ILAE type IIId.

Astrocytic tumour grading: a comparative study of three-dimensional pseudocontinuous arterial spin labelling, dynamic susceptibility contrast-enhanced perfusion-weighted imaging, and diffusion-weighted imaging.

OBJECTIVES: We hypothesized that three-dimensional pseudocontinuous arterial spin labelling (pCASL) may have similar efficacy in astrocytic tumour grading as dynamic susceptibility contrast-enhanced perfusion-weighted imaging (DSC-PWI), and the grading accuracy may be further improved when combined with apparent diffusion coefficient (ADC) values. METHODS: Forty-three patients with astrocytic tumours were studied using diffusion weighted imaging (DWI), pCASL, and DSC-PWI. Histograms of ADC and normalized tumour cerebral blood flow values (nCBF on pCASL and nrCBF on DSC-PWI) were measured and analyzed. RESULTS: The mean 10 % ADC value was the DWI parameter that provided the best differentiation between low-grade astrocytoma (LGA) and high-grade astrocytoma (HGA). The nCBF and nrCBF (1.810 ± 0.979 and 2.070 ± 1.048) in LGA were significantly lower than those (4.505 ± 2.270 and 5.922 ± 2.630) in HGA. For differentiation between LGA and HGA, the cutoff values of 0.764 × 10(-3) mm(2)/s for mean 10 % ADC, 2.374 for nCBF, and 3.464 for nrCBF provided the optimal accuracy (74.4 %, 86.1 %, and 88.6 %, respectively). Combining the ADC values with nCBF or nrCBF could further improve the grading accuracy to 97.7 % or 95.3 %, respectively. CONCLUSIONS: pCASL is an alternative to DSC-PWI for astrocytic tumour grading. The combination of DWI and contrast-free pCASL offers a valuable choice in patients with risk factors. KEY POINTS: • pCASL shows positive correlation with DSC-PWI in astrocytic tumour grading. • ADC values based on ADC histograms can be an objective method. • Combination of DWI and pCASL or DSC-PWI can improve grading accuracy.

Operative management of trigeminal neuromas: an analysis of a surgical experience with 55 cases.

BACKGROUND: The trigeminal schwannoma is the second most common intracranial schwannoma. Their proximity to the critical skull base neural and vascular structures increases the complexity of surgical treatment. The aim of this study was to better understand the surgical approaches and the prognosis, as well as to assess the optimum therapeutic schedule. METHODS: This was a retrospective study of 55 patients with trigeminal schwannomas who visited our department between Jan 2007 and Jan 2012. We analyzed the clinical and radiological presentation, tumor characteristics, surgical approaches, the prognosis. RESULTS: The patients were 30 women and 25 men of mean age 36 years (range, 6-66 years) who received postoperative neurological and neuroradiological follow-up. The tumor was located in the middle fossa (type A) in 13 cases, in the posterior fossa (type B) in ten cases, in the middle and posterior fossae (type C) in 21 cases, and in the branches of the trigeminal nerve (type D) in 11 cases. The most common symptom was facial hypesthesia or numbness in 36 patients (65 %) . Total and nearly total tumor resection was achieved in 51 cases (93 %). Three patients (5 %) had worsening of preexisting deficits and there was no perioperative mortality. With an average follow-up period of 35 months, facial hypesthesia persisted in 26 patients (72 %),and improved in ten patients (28 %). Facial pain was relieved in 11 patients (100 %). There has been a recurrence in one case (2 %) and all patients resumed independent and social reintegration. CONCLUSION: This study demonstrates radical surgery with excellent neurological outcomes is the primary treatment of trigeminal schwannomas. Appropriate selection of surgical approach according to tumor types is highly important and necessary. The preoperative facial pain could be relieved, hypesthesia frequently remains or could even be worsened after surgery.

[Leukoencephalopathy with cerebral calcification and cysts: a case report and review of literature].

OBJECTIVE: To improve the diagnostic ability of leukoencephalopathy with cerebral calcifications and cysts (LCC), a rare central nervous system disease. METHODS: The clinical manifestations, neuroimages and neuropathological features of a 19-year-old male patient were analyzed. A total of 20 cases from 14 literatures were reviewed. RESULT: The patient was admitted with right limb weakness, cognitive decline, headache and blurred eyesight. Head CT scan showed multiple calcifications, cysts formation and leukoencephalopathy. Brain MRI showed several cysts in bilateral hemisphere, basal ganglia, thalamus and paraventricular areas. A mural nodule was noted inside one of the cyst, which was enhanced on the contrasted MRI. The wall of the cysts was partially enhanced, but not with the fluid inside the cysts. The corresponding CT calcifications foci showed on T1 and T2 with either both hyperintensity or both hypointensity, which was also partial enhanced. Extensive leukoencephalopathy was formed around the cysts and the ventricles. But neither Cho nor NAA changed a lot on MRS. Amplitude diagram of SWI series exhibited multiple round small dark signals all over the affected areas with mixed signals showed in the phase diagram, which indicated both calcifications and microbleeding at the lesions. Neuropathological examinations found no tumor cells in the operated cyst, and showed angiomatous small blood cells were dominant in the cyst wall. Hyaline degenerations, microcalcifications and hemosiderin deposition were observed. No obvious demyelination was discovered, while gliosis, numerous Rosenthal fibers and fibrinoid vascular necrosis were found around the lesions. The clinical, neuroimaging and pathological features of this patient were in accordance with the cases reported in the literatures. CONCLUSIONS: Neuroimaging is the most important method for the diagnosis of LCC. As small vessel lesions are probably closely related to the pathophysiology of LCC, SWI could be recommended to further reveal the etiology of LCC.

[Pathologic features of ulegyria in refractory epilepsy by modified anatomic hemispherectomy: a clinicopathologic study of 39 cases].

OBJECTIVE: To investigate the clinicopathologic features of the brain tissue diagnosed as ulegyria from modified anatomic hemispherectomy for refractory epilepsy. METHODS: The clinical and pathologic findings were reviewed in 39 patients who underwent modified anatomic hemispherectomy and diagnosed as ulegyria in the Epilepsy Center of Tsinghua University Yuquan Hospital from 2007 to 2011. RESULTS: All patients including 30 males and 9 females had medically intractable seizures, and the mean age of seizure onset and disease duration were 4.0 years and 7.3 years respectively. Significant history included febrile seizure in 14 patients (35.9%), cerebral hemorrhage in 8 patients (20.5%), fetal distress and surgical trauma each in 6 patients (15.4%), vascular malformation and cerebral hemorrhage in 1 patient (2.6%), and unclear history in 4 patients (10.2%). Histologically, all cases were characterized by cortical destruction, with neuronal loss and gliosis. All cases were accompanied by varying degree of cortical dysplasia, which were diagnosed as focal cortical dysplasia IIId. Hippocampus sclerosis was identified in 2 cases. Seizure outcome after surgery revealed 37 patients (94.9%) had an Engel grade I, two patients (5.1%) had an Engel grade II. CONCLUSIONS: Febrile seizure, cerebral hemorrhage, fetal distress and surgical trauma in childhood can lead to refractory epilepsy. Histopathological change in the brain is ulegyria accompanied by focal cortical dysplasia IIId. Modified anatomic hemispherectomy is an effective therapy to treat those patients with extensive changes of one hemisphere.

Metagenomic Next-Generation Sequencing of Cerebrospinal Fluid for the Diagnosis of Cerebral Aspergillosis.

Purpose: Cerebral aspergillosis (CA) is a rare but often fatal, difficult-to-diagnose, opportunistic infection. The utility of metagenomic next-generation sequencing (mNGS) for diagnosis of CA is unclear. We evaluated the usefulness of mNGS of the cerebrospinal fluid (CSF) for the diagnosis of CA. Methods: This prospective study involved seven consecutive patients with confirmed CA in whom CSF mNGS was performed. Serum (1→3)-ß-D-glucan and galactomannan levels were determined, and histopathological examination and mNGS of the CSF were conducted. CSF specimens from three non-infected patients were used as positive controls. Results: mNGS of the CSF was positive in six of the seven confirmed CA cases (85.71% sensitivity). In the cryptococcal meningitis group (control), mNGS of the CSF was positive for Aspergillus in two patients (84.62% specificity). The positive likelihood ratio, negative likelihood ratio, and Youden's index of mNGS for CA in the CSF were 5.565, 0.169, and 0.7, respectively. Among the six mNGS-positive cases, more than two Aspergillus species were found in four (4/6, 66.67%). In the positive controls, the addition of one A. fumigatus spore yielded a standardised species-specific read number (SDSSRN) of 25.45 by mNGS; the detection rate would be 0.98 if SDSSRN was 2. Conclusion: mNGS facilitates the diagnosis of CA and may reduce the need for cerebral biopsy in patients with suspected CA. Trial Registration Number: Chinese Clinical Trial Registry, ChiCTR1800020442.

Cerebellopontine angle neurenteric cyst with focal malignant features.

Neurenteric cysts are uncommon cystic lesions lined by endodermal-derived epithelium, which are rarely found in the CNS, especially in the intracranial region. Although recurrences and disseminations of these cysts have been reported, only one case of intracranial malignant transformation has previously been described. Here we report a cerebellopontine angle neurenteric cyst in a 26-year-old woman. The cyst wall was lined by columnar epithelium with atypical nuclei and high MIB-1 index. In addition, stromal invasion was found in the subepithelial areas, which shows malignant features. Dissemination was speculated on MRI 6 months after total excision of the original cyst.

Diffuse Leptomeningeal Glioneuronal Tumor Presented with Language Developmental Delay.

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a new central nervous system tumor defined by the WHO in 2016 characterized mainly by hydrocephalus, headache, and epilepsy. We reported the case of a patient diagnosed with DLGNT who presented with language developmental delay and positive but subtle changes in imaging two years before the emergence of typical clinical symptoms. Few studies were conducted on early tumor symptoms due to the limited number of cases. We hypothesized that with the existence of pre-tumor symptoms, language developmental delay may be related to tumor.

Optimizing the Extent of Resection and Minimizing the Morbidity in Insular High-Grade Glioma Surgery by High-Field Intraoperative MRI Guidance.

AIM: The result of surgical resection for insular high-grade glioma (HGG) is disappointing due to the complex anatomy of insula and aggressive growth of HGG. The use of intraoperative magnetic resonance imaging (iMRI) was reported to improve the results of glioma surgery. The authors aimed to evaluate the impact of high-field iMRI and functional neuronavigation on the surgical resection of insular HGG. MATERIAL AND METHODS: Between July 2010 and July 2014, 51 insular HGG patients underwent operations guided by combined high-field iMRI and functional neuronavigation. Twenty-two insular HGG patients underwent conventional neuronavigation operations were assessed as the control group. Preoperative and postoperative tumor volumetric scan analysis, Karnofsky performance score (KPS) and follow-up results were reviewed retrospectively. RESULTS: Residual tumor was detected by the iMRI in 42 patients, and residual tumor of 37 patients was further resected in the iMRI-assisted group. The median extent of resection (EOR) increased significantly from 79% (58%?100%) to [96% (86%? 100%), p < 0.001]. The median EOR of iMRI-assisted group [96% (86%?100%)] was significantly higher than that of conventional neuronavigation group [84% (69%?100%); p=0.031]. Mean residual tumor volume of iMRI-assisted group [0.6 (0.0-5.2) cm3] was significantly smaller than that of conventional neuronavigation group [3.8 (0.0-12.1) cm3; p=0.003]. KPS within 3 days after surgery reduced and KPS at 3 months after surgery improved for both groups. KPS of iMRI-assisted group [90(70-100)] was significantly higher than that of control group [80(60-100); p=0.021] at 3 months after surgery. The median progression-free survival (PFS) of iMRI-assisted group [18(9-42) months] was better than that of control group [15(3-32) months; p=0.010]. The median overall survival (OS) of iMRI-assisted group [28(14-49) months] was better than that of control group [18 (7-38) months; p=0.035]. CONCLUSION: Combined high-field iMRI and functional neuronavigation optimize the extent of resection and minimize the morbidity in insular HGG surgery. Aggressive resection of insular HGG is predictive of improved OS and PFS.

[Clinicopathologic and radiologic features of dysembryoplastic neuroepithelial tumors].

OBJECTIVE: To study the clinicopathologic features and radiologic findings of dysembryoplastic neuroepithelial tumor (DNT). METHODS: The clinical presentations, radiologic findings, histologic features and immunophenotype of 9 cases of DNT were analyzed. RESULTS: The age of patients ranged from 12 to 51 years (mean age = 32 years). Most presented with partial seizures, sometimes accompanied by transient aphasia, agraphia and decreased visual acuity. One case was asymptomatic and discovered incidentally during a routine check-up. All patients had no neurological deficit found on physical examination. All tumors were located in the supratentorial cerebral cortex. There was no peritumoral edema or space-occupying effect on radiologic examination. The tumors involved either frontal lobe (number = 4), temporal lobe (number = 4), frontoparietal lobe (number = 1) . Two cases showed cystic changes. Two histologic variants of DNT were recognized: simple (number = 3) and complex (number = 6). Simple variant was composed mainly of the glioneuronal element, accompanied by surrounding oligodendrocyte-like cells, and the complex variant contained a low-grade glioma component, in addition to the glioneuronal element and sometimes foci of cortical dysplasia. CONCLUSIONS: DNT is a benign tumor with excellent prognosis after surgical excision. Local recurrence is rare. Complex variant of DNT needs to be distinguished from other types of low-grade glioma.

Multimodal treatment and management strategies for intracranial hemangiopericytoma.

Intracranial hemangiopericytoma (HPC) is a rare malignant meningothelial tumor. The authors retrospectively reviewed the long-term clinical outcomes of patients with HPC with regard to treatment modalities and histopathological grades. Eighteen women and 20 men (mean age 38.5 years, range, 18-62 years) were observed over an average follow-up period of 61 months (range, 15-133 months) between 2003 and 2013. The initial treatment modalities included total tumor resection followed by conventional radiotherapy (RT) (n=27), and subtotal tumor resection followed by stereotactic radiosurgery (n=11). One patient (3%) had permanent neurological deficits, and six patients (16%) died. Thirteen patients (34%) suffered recurrence. One year, 5 year, and 10 year recurrence-free survival rates were 100%, 70%, and 39%, respectively. Five patients (13%) developed metastasis. One year, 5 year, and 10 year metastasis-free survival rates were 100%, 89%, 74%, respectively. Low grade tumors were associated with longer overall survival, recurrence-free interval and metastasis-free interval (log-rank, p<0.05). Radical resection with RT was associated with longer overall survival and recurrence-free interval (log-rank, p<0.05), but had no effect on the metastasis-free interval (log-rank, p=0.245). Thus, radical surgery followed by adjuvant RT is the primary treatment of HPC, but recurrence and metastasis remain a common treatment outcome regardless of initial strategy. It is necessary to maintain long-term follow-up and serial imaging for all patients with intracranial HPC after treatment, regardless of extent of resection.

Sturge-Weber Syndrome Is Associated with Cortical Dysplasia ILAE Type IIIc and Excessive Hypertrophic Pyramidal Neurons in Brain Resections for Intractable Epilepsy.

Sturge-Weber syndrome (SWS) is a rare syndrome characterized by capillary-venous malformations involving skin and brain. Many patients with SWS also suffer from drug-resistant epilepsy. We retrospectively studied a series of six SWS patients with epilepsy and extensive neurosurgical resections. At time of surgery, the patients' age ranged from 11 to 35 years (with a mean of 20.2 years). All surgical specimens were well preserved, which allowed a systematic microscopical inspection utilizing the 2011 ILAE classification for focal cortical dysplasia (FCD). Neuropathology revealed dysmorphic-like neurons with hypertrophic cell bodies reminiscent to those described for FCD type IIa in all cases. However, gross architectural abnormalities of neocortical layering typical for FCD type IIa were missing, and we propose to classify this pattern as FCD ILAE type IIIc. In addition, our patients with earliest seizure onset also showed polymicrogyria (PMG; n = 4). The ictal onset zones were identified in all patients by subdural electrodes, and these areas always showed histopathological evidence for FCD type IIIc. Four out of five patients had favorable seizure control after surgery with a mean follow-up period of 1.7 years. We concluded from our study that FCD type IIIc and PMG are frequently associated findings in SWS. FCD type IIIc may play a major epileptogenic role in SWS and complete resection of the associated FCD should be considered a prognostic key factor to achieve seizure control.

[An analysis of the causes of dementia in 383 elderly autopsied cases].

OBJECTIVE: To survey the causes of dementia confirmed by autopsy in the elderly and to have a better understanding of various causes of senile dementia. METHODS: A retrospective study on clinical and pathological diagnosis in 383 cases aged 60 and over with autopsy performed was carried out clinical diagnosis of dementia was based on DSM-IVR, NINCDS-ADRDA and NINCDS-AIREN criteria. Gallyas-Braak staining, Tau and Ubiquitin Immunohistochemistry staining were adopted for diagnosing neurodegenerative dementia and distinguishing various degenerative diseases. RESULTS: Among the 383 aged cases with consecutive autopsy, 78 cases were found to have clinical features and brain pathologic change related to dementia. The incidence of dementia in the aged patients with autopsy was 20.4%. Of all the cases, vascular dementia accounted for 38.5% (30 cases), being the most frequently on countered. Next in order were degenerative dementias including Alzheimer's disease (11 cases) and non-Alzheimer's degenerative dementia (9 cases) including dementia with Lewy bodies, corticobasal degeneration, progressive supranuclear palsy, Pick's disease and idiopathic Parkinson'disease. Degenerative dementias accounted for 25.6% (20 cases). Various medical disorders including hepatic encephalopathy, pulmonary encephalopathy, hypoglycemia with cognitive impairment were also relatively common, they accounted for 20.5% (16 cases). The other less common causes included brain tumor, normal pressure hydrocephalus and subdural hematoma, 12 cases accounted 15.4%. The total accordance rate of clinical and pathologic diagnosis of senile dementia was 64.5%, the accordance rates of medical disorders such as hepatic encephalopathy, pulmonary encephalopathy and brain metastatic tumors were all 100%, the accordance rate of vascular dementia and degenerative dementia was 66.7% and 40% respectively. CONCLUSION: Senile dementia is a clinical syndrome caused by multiple factors. Physician should think of various possible causes of dementia, basing on their clinical history and laboratory results. Promoting autopsy and adopting new pathological techniques would increase the diagnostic accuracy of degenerative dementia.

[Glial abnormalities in progressive supranuclear palsy and corticobasal degeneration].

OBJECTIVE: To study pathologic features of glial cells in progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) and to explore their pathologic significance. METHODS: Brain tissues from 2 cases with PSP and 3 cases with CBD, all confirmed by autopsies, were examined by routine neuropathologic methods, Gallyas-Braak staining and tau immunostaining. Brain tissues from 6 Alzheimer's disease cases, 4 cases with Parkinson's disease and 6 elderly with no neurologic abnormality were used as controls. RESULTS: Gallyas-Braak staining demonstrated tuft-shaped astrocytes and coiled-body oligodendroglial cells in the brain tissues of 2 cases with PSP and 3 cases with CBD. The tuft-shaped astrocytes appeared prominently in the frontal and parietal cortex, basal ganglia and grey matter of the brainstem. The coiled-body oligodendroglial cells were distributed widely in the white matter of the frontal and parietal lobes, basal ganglia, brainstem and cerebellum. However, astrocytic plaques, composed of degenerative stubby processes with radiating arrangement, only appeared in the frontal, parietal and cingular cortex, as well as in the striatum of 3 cases with CBD. The astrocytic plaques and tuft-shaped astrocytes coexisted in the same areas, including parietal and cingular cortex and striatum, in CBD. All these glial abnormalities showed tau-positive immunoreaction not found in control cases. CONCLUSIONS: The tuft-shaped astrocytes and coiled-body oligodendroglial cells are common glial morphologic features of both PSP and CBD. Astrocytic plaques are also characteristically seen in CBD.

[Morphological and quantatitive capillary changes in aging human brain].

OBJECTIVE: To investigate morphological changes of capillary in aging brain and explore the role of vascular factor in brain aging. METHODS: Twenty-eight brains of individuals (mean age 65 years) who died without clinical or pathological involvement of nervous system and 6 brains of Alzheimer's disease (AD) patients (mean age 83 years) were obtained at autopsy. Sections from frontal lobe, occipital lobe, striatum and hippocampus of normal subjects and sections from hippocampus of AD patients were used for hematoxylin eosin (HE), lox fast blue (LFB), toluidine blue stains and ulex europaeus agglutinin (UEA) immunostaining. After observations of morphological changes of neuron and capillary, computer-aid image analysis was performed to quantify numerical density and area density of neuron and capillary in frontal lobe, occipital lobe, putamen, CA3 sector of normal subjects and CA3 sector of AD patients. Numerical ratio and area ratio of neuron and capillary were then calculated. Correlations between neuron/capillary ratio and age were estimated using Pearson's correlation test. Difference of neuron/capillary ratio in CA3 sectors between AD patients and advanced aged normal subjects (> 75 years) was analyzed with Student's t-test. RESULTS: Several pathological microvascular changes, including increased tortuosity, looping, bundling, stringing, and effacement of endothelia were seen in aged subjects and more prevalent in AD patients. Numerical ratio and area ratio of neuron and capillary of frontal lobe, occipital lobe and putamen significantly increased with age in normal aging subjects. CONCLUSIONS: Morphological changes and relative decrease in number and capacity of capillary in aging brain may reduce cerebral blood flow and metabolism, and consequently result in functional impairment of aging brain. Vascular factors may play an important role in the development of brain aging.

Wernicke's encephalopathy in nonalcoholic patients: clinical and pathologic features of three cases and literature reviewed.

Three cases of Wernicke's encephalopathy in nonalcoholic patients diagnosed by postmortem examination were reported to improve the recognition of this disease. All three cases were male, ages ranged from 33 to 73 years old. All the cases had a clinical history of malnutrition but no history of chronic alcoholism. Routine autopsy and neuropathologic investigations examining the histological changes of the brain were performed. Pathological findings included recent petechial and local hemorrhages in the mamillary bodies, periventricular regions around the third and fourth ventricles and aqueduct. Under light microscopy the proliferation and dilatation of the capillaries was particularly prominent in the mamillary bodies and pericapillary hemorrhages were present in the periventricular regions. Neuronal losses were found only in the medial nucleus of the thalamus and inferior olive, myelin staining demonstrated demyelination and gliosis in those areas. The diagnosis of Wernicke's encephalopathy was made. In combination with the reviewed literature, our cases suggest that Wernicke's encephalopathy can occur not only in patients with alcohol abuse, but also in those who have suffered thiamine deficiency due to metabolic and nutritional disorders.