Hypothermia Presenting in Wernicke Encephalopathy: A Case Report

Wernicke encephalopathy (WE) is a neurologic disorder characterized by clinical symptoms, such as nystagmus, ataxia, and mental confusion. Hypothermia in patients with WE is a rare complication, and its pathogenic mechanism and therapy are yet to be ascertained. Herein, we presented a case of a 61-year-old man who was diagnosed with WE 3 months earlier. We investigated the cause of hypothermia (35.0℃) that occurred after an enema (bowel emptying). Brain magnetic resonance imaging revealed mammillary body and hypothalamus atrophy. In the autonomic function test, the sympathetic skin response (SSR) test did not evoke SSR latencies on both hands. In addition, abnormal orthostatic hypotension was observed. Laxative and stool softener medication were administered, and his diet was modified, which led to an improvement in constipation after 2 weeks. Moreover, there was no recurrence of hypothermic episode. This is the first reported case of late-onset hypothermia secondary to WE.

Nonalcoholic Wernicke's Encephalopathy With Diffuse Cortical Involvement

Wernicke's encephalopathy is an acute neurological deterioration due to a reversible brain lesion caused by thiamine deficiency. Most of the affected patients are thiamine-depleted alcoholics, and the condition usually involves the medial thalami, mammillary bodies, and periaqueductal area. However, there are rare reports of lesions in the cerebellum and cerebral cortex, especially in patients with nonalcoholic Wernicke's encephalopathy. We report a case of nonalcoholic Wernicke's encephalopathy involving atypical diffuse cortical regions, and review previously reported cases.

A Case of Acute Incomitant Esotropia Associated with Wernike's Encephalopathy

PURPOSE: We report a case of an acute incomitant esotropia with Wernike's encephalopathy. CASE SUMMARY: A 64-year-old male visited our hospital because of diplopia lasting a week. He was a chronic alcoholic drinking two bottles of makgeolli daily and eating little for a month. He showed -2 underaction of bilateral lateral rectus muscles and 45 prism diopters of esotropia at the primary position at the first visit. He had ataxia and mild cognitive impairment. There were high signal intensities in the periaqueductal area and mammillary bodies in the brain fluid attenuated inversion recovery magnetic resonance image. He was diagnosed with Wernike's encephalopathy clinically and was immediately treated with intravenous thiamine. He showed -0.5 underaction of bilateral lateral muscles and 8 prism diopters of esotropia at the primary position 3 days after thiamine treatment. CONCLUSIONS: Wernicke's encephalopathy is a medical emergency. If diagnosis and treatment are delayed, patients may have neurological sequelae that can lead to death. Esotropia and diplopia can be the presenting manifestations in Wernike's syndrome without other symptoms. In taking patient histories, physicians should ask about alcohol consumption and low food intake because of the possibility of acute incomitant esotropia associated with Wernicke's encephalopathy.

Morfometría del cuerpo calloso y de los cuerpos mamilares en el alcoholismo por resonancia magnética / Morphometry of the corpus callosum and mammillary bodies in alcoholism using magnetic resonance

Las alteraciones morfológicas encefálicas en individuos que padecen alcoholismo crónico explican los severos trastornos neurocognitivos, principalmente amnésicos anterógrados, y neuropsiquiátricos debido al carácter neurotóxico y déficits nutricionales que genera. El objetivo fue describir la morfología del cuerpo calloso y los cuerpos mamilares en dependientes al alcohol y su relación volumétrica otras áreas cerebrales. Se estudiaron morfológica y morfométricamente con resonancia magnética los cerebros de 26 masculinos, edades 29 a 51 años, con diagnóstico de trastorno por consumo de alcohol tipo dependencia (DSM IV). Fueron criterios de exclusión: patologías hepáticas, autoinmunes, neurológicas centrales y antecedentes neurológicos no relacionados con el consumo, traumatismo craneoencefálico o neurocirugías previas. Se cuantificó la volumetría (método ROI) de: corteza prefrontal, hipocampo, cuerpo amigdalino, tálamo y cuerpos mamilares; y la morfometría bidimensional de las regiones del cuerpo calloso (métodos de Witelson y de Clarke). El procedimiento fue sometido a pruebas estadísticas y cumplió con los reparos ético-legales vigentes. Todos los volúmenes de pacientes dependientes fueron menores. Se registró según severidad reducción volumétrica significativa en corteza prefrontal, cuerpo mamilar, hipocampo y tálamo izquierdos, seguidos por el cuerpo amigdalino derecho. La superficie con mayor afectación del cuerpo calloso fue el 1/3 anterior. Se determinaron correlaciones entre la afectación de los cuerpos mamilares y el cuerpo calloso con estructuras subcorticales. El compromiso del cuerpo calloso y los cuerpos mamilares presentó correlación con la afectación del hipocampo, cuerpo amigdalino y tálamo, y una estrecha relación con el tiempo y cantidad de consumo. Estas alteraciones morfométricas son indicadores de severidad cognitiva y alteración comportamental.

Brain morphological abnormalities in individuals with severe chronic alcoholism explains the severe neurocognitive disorders, namely neuropsychiatric and anterograde amnesia, due to the neurotoxic nature and nutritional deficits generated. The objective of the present study was to describe in alcohol dependent subjects, the morphology of the corpus callosum and mammillary bodies and volume ratio in other brain areas. We used morphological and morphometric magnetic resonance imaging to study the brains of 26 men, ages 29-51 years diagnosed with alcohol dependence type disorder (DSM IV). Exclusion criteria were: liver diseases, autoimmune and central neurological disorders, as well as a neurological history not related to consumption, prior traumatic brain injury or brain surgery. Volumes were quantified by (ROI method) for the following: prefrontal cortex, hippocampus, amygdaloid body, thalamus and mammillary bodies, and the two-dimensional morphometry of corpus callosum regions (Witelson and Clarke methods). The procedure was subject to statistical tests and current legal ethical standards. Our results showed all patients dependent volumes were lower. In accordance with severity, significant volume reduction was recorded in prefrontal cortex, mammillary body, left hippocampus and thalamus, followed by the right amygdaloid body. The most affected area of the corpus callosum was the anterior third. Correlations were determined between the involvement of the mammillary bodies and the corpus callosum with subcortical structures. The relation of the corpus callosum and mammillary bodies correlated with the involvement of the hippocampus, amygdala and thalamus body, and are closely related to the amount of time and consumption of alcohol. These morphometric changes are indicative of severe cognitive and behavioral impairment.

A Case of Thiamine (Vitamin B1)-Deficient Optic Neuropathy Associated with Wernicke's Encephalopathy

PURPOSE: To report a case of subacute optic neuropathy caused by thiamine (vitamin B1) deficiency. CASE SUMMARY: A 44-year-old woman was referred to the ophthalmology department due to decreased vision which began 10 days prior to presentation. The patient history indicated that she had undergone chemotherapy for ovarian cancer and she had been dependent on total parenteral nutrition for 3 weeks due to nausea and vomiting. Her best corrected vision of the right and the left eyes were 0.15 and 0.2, respectively. Color vision was severely impaired in both eyes without retrobulbar pain. There was marginal blurring on the temporal side of the optic discs of both eyes. The optic nerves were unremarkable on orbital and brain magnetic resonance imaging (MRI). There was high signal intensities in both the mammillary body and periaqueductal gray matter on T2-weighted imaging. In addition, the patient exhibited ataxia along with short-term memory loss. She was diagnosed with Wernicke's encephalopathy. Thiamine was administrated based on the diagnosis, and after 2 days of administration, the patient's vision and neurologic symptoms began to improve. Two weeks later, the patient recovered her vision. CONCLUSIONS: Thiamine deficiency may cause optic neuropathy and can be recovered with early thiamine supplementation. This condition could occur due to deficient oral thiamine administration. We should keep this point in mind and try to prevent or diagnose early.

Case of Atypical Wernicke's Encephalopathy in a GB Cancer Patient / 대한내과학회지

Wernicke's encephalopathy (WE) is an acute neuropsychiatric syndrome resulting from thiamine deficiency. Traditionally, diagnosis of WE rests on a clinical symptom triad consisting of ocular signs, altered consciousness, and ataxia. However, the complete triad is only present in a fraction of cases, which means that WE tends to be under-diagnosed, especially in nonalcoholic patients. Brain MRI of WE patients usually shows symmetrical signal intensity alterations in the thalami, mammillary bodies, and periaqueductal area, because of cytotoxic edema in the same region. These typical findings are useful diagnostic leads in WE patients with atypical symptoms. However, atypical findings can occasionally be seen in the vermis of cerebellum and cerebellar nuclei. Notably, alterations of signal intensity in the cerebellar dentate nuclei, which is a typical finding of metronidazole-induced encephalopathy (MIE), need to be distinguished according to medication history and response to thiamine.

Papez circuit: An anatomical study by cadaveric dissection

This study was done to completely the Papez circuit by cadaveric dissection and it's relation to ventricles and other related anatomical structures. Eight formalin fixed cerebral hemispheres were microscopically dissected for Papez circuit. Klingler's technique of fibre dissection was adopted. The circuit was dissected from medial and superior-lateral cerebral surfaces. Bilateral hemispheric dissection was done simultaneously in intact brain from superior-lateral surface in two brains [4 hemispheres]. During and after dissection it's relation with lateral ventricles and other related structures were studied. Papez circuit was demonstrated by total dissection. Fibres leave the hippocampal formation and proceed through the fomix; most of these fibres have been shown to terminate in the mammillary nuclei of the hypothalamus. From here, the mammillothalamic tract ascends to the anterior group of thalamic nuclei. This group of nuclei projects to the cingulated gyrus through the anterior limb of internal capsule to anterior cingulated gyrus. From the cingulated gyrus there is an association bundle; the cingulum, which connects the cingulated gyrus with the parahippocampal gyrus part of the limbic lobe. The parahippocampal gyrus projects to the hippocampal formation and circuit is completed. Relation of different parts of the circuit with surrounding structures were also clearly seen. Knowledge of the microsurgical anatomy of the Papez circuit is not only important for understanding memory mechanism and other limbic functions but also very important in management of lateral and third ventricular lesions, in transcallosal, transventricular, supraseller and temporal lobe surgery, and for psycho-neurosurgery

The Variable Ellipsoid Modeling Technique as a Verification Method for the Treatment Planning System of Gamma Knife Radiosurgery

OBJECTIVE: The secondary verification of Leksell Gamma Knife treatment planning system (LGP) (which is the primary verification system) is extremely important in order to minimize the risk of treatment errors. Although prior methods have been developed to verify maximum dose and treatment time, none have studied maximum dose coordinates and treatment volume. METHODS: We simulated the skull shape as an ellipsoid with its center at the junction between the mammillary bodies and the brain stem. The radiation depths of the beamlets emitted from 201 collimators were calculated based on the relationship between this ellipsoid and a single beamlet expressed as a straight line. A computer program was coded to execute the algorithm. A database system was adopted to log the doses for 31x31x31 or 29,791 matrix points allowing for future queries to be made of the matrix of interest. RESULTS: When we compared the parameters in seven patients, all parameters showed good correlation. The number of matrix points with a dose higher than 30% of the maximal dose was within +/- 2% of LGP. The 50% dose volume, which is generally the target volume, differs maximally by 4.2%. The difference of the maximal dose ranges from 0.7% to 7%. CONCLUSION: Based on the results, the variable ellipsoid modeling technique or variable ellipsoid modeling technique (VEMT) can be a useful and independent tool to verify the important parameters of LGP and make up for LGP.

Postnatal developmental and aging changes of the mammillary body in the albino rats [histological and ultrastructural study]

The mammillary body is the most prominent structure in the caudal hypothalamus of rats as it is involved in a variety of associative and memory processes. The aim of this study was to throw light on the postnatal developmental and aging changes of the mammillary body nuclei in the rat. A total number of 65 albino rats were used in this work. The age groups of the animals included new born, fifteen days, one month, two months and two years old rats. Specimens were processed for ordinary histological study and stained with Gallocyanin chrom-alumstain and Golgicox method. Ultrastructural study with the aid of the transmission electron microscope was done for the adult and aged rats. The results of this study revealed that the mammillary body is composed of a lateral and a medial mammillary nucleus, the latter has been subdivided into four subnuclei: pars lateralis, pars medianus, pars medialis, and pars posterior. The cells are either medium-sized or small and the sizes differ, among different subnuclei. The largest cell sizes are found in the lateral mammillary nucleus. These cells have long branching dendrites that are often beaded with no spines. Most cells in the medial mammillary nucleus are characterized by long branching dendrites and exhibit numerous spines. Cells of the mammillary nuclei are characterized by closely apposed neuronal somata and imaginations of the nuclear membrane. There are also various types of axosomatic synaptic terminals with the mammilary neurons. The neurons of the mammillary nuclei showed increase in the amount of Nissl material, progressive increase in thickness, extension and branching of their dendrites with development, there is also apparent increase in the size of the mammillary body. In the aged rat, there are some degenerative changes in the mammillary neurons in the form of apparent reduction in the size of the mammillary body relative to the adult age. There is decreased staining affinity of the cytoplasm, extensive decrease in the thickness and extension of the dendrites with decrease in the amount of somatic and dendritic spines. Most neurons reveal progressive accumulation of lipofuscin pigment and marked imaginations of the nuclear membrane with the progress of age. As the mammillary body is involved mainly in memory, this function could be expected to decline with age as a consequence of these degenerative changes

Wernicke Encephalopathy in a Patient with Long-Standing Vomiting after Pancreatoduodenectomy / 신경정신의학

Wernicke encephalopathy is an acute neurologic disorder attributable to thiamine (vitamin B1) deficiency. We report the case of a 61-year-old female patient who presented Wernicke encephalopathy after surgery for pancreatic head cancer. From the ninth postoperative day, she had suffered from nausea and vomiting and had difficulties ingesting food, she was given total parenteral nutrition (TPN), but lacked adequate vitamin (thiamine) supplementation. After 28 days, she developed ataxia, ophthalmoplegia, and mental confusion. The magnetic resonance image showed pathologic changes in the medial thalamus, periaqueductal gray matter, medulla and mamillary bodies. The blood level of thiamine was very low. After intravenous and oral supplementation of thiamine (200 mg/day), consciousness was soon normalized and neurologic symptoms have gradually been improving. Nausea and vomiting disappeared after administration of a low dose of mirtazapine (7.5 mg/day). We emphasize the importance of thiamine supplementation to the patients who suffer from vomiting which hinders them from taking food and who require prolonged TPN.

Amnesic Syndrome in a Mammillothalamic Tract Infarction

It is controversial whether isolated lesions of mammillothalamic tract (MTT) produce significant amnesia. Since the MTT is small and adjacent to several important structures for memory, amnesia associated with isolated MTT infarction has been rarely reported. We report a patient who developed amnesia following an infarction of the left MTT that spared adjacent memory-related structures including the anterior thalamic nucleus. The patient s memory deficit was characterized by a severe anterograde encoding deficit and retrograde amnesia with a temporal gradient. In contrast, he did not show either frontal executive dysfunction or personality change that is frequently recognized in the anterior or medial thalamic lesion. We postulate that an amnesic syndrome can develop following discrete lesions of the MTT.

Analysis of parahippocampal gyrus in 115 patients with hippocampal sclerosis

PURPOSE: Analysis of the parahippocampal gyrus (PHG) involvement in 115 patients with hippocampal sclerosis (HS) by MR imaging. The simultaneous occurrence of ipsilateral fornix (F) and mamillary body (MB) volume loss was checked also. These findings were correlated with the side of hippocampal involvement, the sex, patient s age, and the symptoms onset. METHOD: The MR images of 115 patients with HS were studied retrospectively. All the examinations were performed on 1.5 T units (SIGNA, GE, Milwaukee, WI) and included high resolution coronal T2-weighted images (3 mm thickness, 0.6 mm gap). RESULTS: The patient's age ranged between 3.5 and 80 years (mean 34.1); 62 (53.9 percent) were female and 53 (46.1 percent) were male. There were HS on the left side in 53 (46.0 percent), on the right side in 51 (44.3 percent), and bilateral in 11 (9.7 percent). In 43 (37.3 percent) cases there were ipsilateral PHG volume loss and signal hyper intensity on T2-weighted imaging. In 29 (25.2 percent) cases there were ipsilateral fornix volume loss and in 10 (34.5 percent) of this there were also ipsilateral MB changes. In abnormal PHG, 23 (53.4 percent) were on the left side, 17 (39.5 percent) were on the right side, and 3 (7.1 percent) were bilateral. There were fornix changes in 15 (34.8 percent) cases and MB volume loss in 5 (11.6 percent) cases. Pertinent clinical data were obtained in only 18 (41.8 percent) of the PHG lesion cases and 11 (61.1 percent) of these patients had epileptic attacks for more than 20 years before the examination. CONCLUSION: PHG involvement must be investigated in patients with HS and we suggest that the term mesial temporal sclerosis should be used only if there are also changes at this anatomical site

A Case of Wernicke's Encephalopathy in a Patient with Multiple System Atrophy

A 41-year-old man was admitted due to altered mentality and confusion. He had showed progressive cerebellar ataxia, dysarthria, gait disturbance from his age of 33 years old. Brain MRI revealed high signal lesions in periaqueductal gray matter, mammillary bodies, median thalami and postcentral gyri bilaterally on T2-weighted images. Severe cerebellar atrophy was noted, too. We report a case of Wernicke's encephalopathy in a patient with probable multiple system atrophy. As far as we know, there have been no published report on this kind of case.

Thalamic Damage in Temporal Lobe Epilepsy with Mesial Temporal Sclerosis / 대한간질학회지

PURPOSE: To investigate the frequency of the thalamic damage and its relationship with clinical data in patients with temporal lobe epilepsy and mesial temporal sclerosis (MTS). METHODS: We evaluated 45 patients with temporal lobe epilepsy showing unequivocal changes of unilateral MTS by the visual inspection of magnetic resonance imaging (MRI) compared to 20 controls. We presumed the inferior margin of the lateral ventricle (IMLV) posterior to the foramen of Monro reflects the volume of the thalamus, and investigated the thalamic damage by comparing bilateral IMLVs on oblique coronal T2 MR images posterior to the foramen of Monro. The relationships between clinical data and the presence of asymmetry of IMLV were evaluated. RESULTS: None of the control group had asymmetry of the hippocampus or IMLV. Asymmetry of IMLV was observed in 42% (19 of 45) of the patient group;IMLV was downward on the ipsilateral side in 40% (18), and on the contralateral side of MTS in one of the four patients having a larger lateral ventricle on the contralateral side of MTS. An asymmetric small fornix and mamillary body was found in 42% and 29% of the patients, respectively, in all ipsilateral to the side of MTS. A history of status epilepticus (SE) was more frequent in patients with downward IMLV ipsilateral to the side of MTS than in patients with symmetry of IMLV (five of 18 versus one of 26, p=0.011). CONCLUSIONS: This study suggests that the thalamic damage may be more frequently associated with MTS, when compared with previous studies, which applied more strict methods in evaluating the thalamic damage. There is a significant correlation between the presence of asymmetrical IMLV and history of SE, and our study suggests that excessive and prolonged seizure activity related to SE, can provoke thalamic injury in patients with temporal lobe epilepsy and MTS.

Gelastic Seizures in a Patient with Tuberous Sclerosis / 대한간질학회지

BACKGROUND: Gelastic seizures are characterized by ictal inappropriate sudden laughter as the predominant seizure manifestation. they are very rare and may occur in patients with hypothalamic hamartomas, pituitary tumors, astrocytomas of the mamillary bodies, CNS infection, trauma, and dysraphic condition. CASE: A 21-year-old woman with tuberous sclerosis was admitted due to frequent sudden inappropriate laughter as a seizure manifestation. EEG showed paroxysmal brief generalized 4-5 Hz spike and wave complexes. Brain MRI revealed multiple tubers in the bilateral cerebral cortical areas, right caudate nucleus, and left anterior cingulate gyrus. We tried vigabatrin, but gelastic seizures were not controlled. COMMENT: We report a case of gelastic seizures associated with tuberous sclerosis, which has not previously been reported in Korea.

Serial MRI Findings in a Clinically Diagnosed Adult Onset Leigh Syndrome

Adult onset Leigh syndrome is a very rare neurodegenerative disorder of unknown cause. We report the evolution of the lesions on serial MRIs in a 38-year-old man with clinically diagnosed Leigh syndrome. We emphasize that the mammillary bodies can be involved during the disease course and that premortem diagnosis of Leigh syndrome is pos-sible, if a characteristic distribution of lesions can be demonstrated on MRI.

MR Imaging Findings of Patients with Mesial Temporal Sclerosis

PURPOSE: To evaluate the MR imaging findings of patients with mesial temporal sclerosis. MATERIALS AND METHODS:We retrospectively reviewed the MR imaging findings of 116 patients diagnosed by MRI as suffering from mesial temporal sclerosis. In 18 of these, the condition was also histologically proven. RESULTS: Among the 116 patients, volume loss of the hippocampus was found in 95 (81.9%) and signal changes of the hippocampus in 53 (45.7%). Decreased signal intensity in the hippocampus on T1-weighted images was found in 13 (11.2%) and increased signal on T2-weighted images in 50 (43.1%). Signal abnormality in the hip-pocampus on both T1- and T2-weighted images was found in ten, and associated extrahippocampal abnormali-ties, as follows, in 20 (17.2%): atrophy of the fornix (n=10), atrophy of the mammillary body (n=8), atrophy of the amygdala (n=10), atrophy or increased T2 signal intensity of the anterior thalamic nuclei (n=2), atrophy of the cingulate gyrus (n=2), atrophy or increased signal intensity of the anterior temporal lobe (n=8), and cere-bral hemiatrophy (n=4). CONCLUSION: A high T2 signal and atrophy of the hippocampus are the most common and important MRI findings of mesial temporal sclorosis. Other abnormal findings, if any, which may be found in extrahippocampal structures such as the fornix, mammillary body and temporal lobe, should, however, also be carefully observed

Reactive Plasticity of Neuroglial Cells on Brain Lesion / 대한해부학회지

Neuroglial cells are actively participate in the pathogenesis or in the recovery procedures following brain lesions. The study was performed to evaluate the plasticity of glial cells following different degree of brain lesions. Neurosurgical operations were made on the rats fixed on the stereotaxic apparatus. Tissue column of 3 mm-diameter was isolated in the caudatoputamen with concomitant severe bleeding in the first group. In the second group, the sensorimotor cortex was suctioned out with moderate bleeding. In the third group, the mammillary body was electrically coagulated with minimal bleeding. Caudatoputamens, as a lesioned tissue or as a target tissue of lesioned area, were studied light and electron microscopically. Observations on reactivities and plasticities of neuroglial cells on the different situations, the following results were obtained : 1. Astrocytes were swollen within an hour following brain lesions. 2. In case of smaller lesion, astroglia alone remove altered structures. 3. Microglia are increased in number, if the lesion is large with severe bleeding. The microglia might come from blood monocyte via transformation to pericyte. 4. In large lesion, astroglia were greatly hypertropied, and microglia might be moving and functioning effeciently within the hypertropied cytoplasm of astroglia. 5. In the stabilizing stage, astroglia produce glial fibers and fix the exhausted microglia. Fixed microglia are proceed into apoptotic process in the cytoplasm of astroglia and removed by them. All these procedures might be controlled by various receptors and secretions of astroglia. It means that astroglia is not only the basic supporting element of nervous tissue, but also an actively functioning element for the most effective homeostatic functioning of the neuropil.