Hematopoietic Stem Cell Transplantation/adverse effects* ; Humans ; Transplantation, Homologous/adverse effects* ; Wernicke Encephalopathy/etiology*

Wernicke's encephalopathy(WE),characterized by nystagmus and ophthalmoplegia,unsteadiness of stance and gait and mental-status changes,is an acute or subacute metabolic encephalopathy of the central nervous system resulting from Vitamin B1(VitB1)deficiency. A 29-year-old male patient was admitted to our hospital due to abdominal pain and fever. He remained chronically undernourished. He was complicated with WE at the late stage of diagnosis,mainly manifested as the convulsion of limbs,ataxia,and delirium. After treatment with VitB1,these neuropsychiatric symptoms were remarkably resolved. His primary disease was later pathologically confirmed as peritoneal mesothelioma.
Adult ; Humans ; Lung Neoplasms ; Male ; Mesothelioma ; Peritoneal Neoplasms ; Thiamine ; Wernicke Encephalopathy

Thiamine (vitamin B₁) is a water-soluble vitamin that is not endogenously synthesized in humans. It is absorbed by the small intestine, where it is activated. Its active form acts as a coenzyme in many energy pathways. We report a rare case of thiamine deficiency in a 3.5-year old boy with short bowel syndrome secondary to extensive bowel resection due to necrotizing enterocolitis during his neonatal age. The patient was parenteral nutrition-dependent since birth and had suffered from recurrent central catheter-related bloodstream infections. He developed confusion with disorientation and unsteady gait as well as profound strabismus due to bilateral paresis of the abductor muscle. Based on these and a very low thiamine level he was diagnosed and treated for Wernicke encephalopathy due to incomplete thiamine acquisition despite adequate administration. He fully recovered after thiamine administration. After 1999 eight more cases have been reported in the PubMed mostly of iatrogenic origin.
Child ; Enterocolitis, Necrotizing ; Gait Disorders, Neurologic ; Humans ; Intestine, Small ; Male ; Parenteral Nutrition, Total ; Paresis ; Parturition ; Short Bowel Syndrome ; Strabismus ; Thiamine Deficiency ; Thiamine ; Vitamins ; Wernicke Encephalopathy

Wernicke encephalopathy is a syndrome caused by thiamine deficiency whose three typical symptoms are ophthalomoplegia, ataxia, and confusion. There are also rare reports of bilateral hearing loss, which can be caused by damage to the bilateral inferior colliculus or thalamic lesions, or by energy failure of the cochlea. This case suggests that thiamine should be administered based on the possibility of Wernicke encephalopathy occurring in malnourished or alcoholic patients with sudden bilateral hearing loss.
Alcoholics ; Ataxia ; Cochlea ; Hearing Loss ; Hearing Loss, Bilateral ; Humans ; Inferior Colliculi ; Thiamine ; Thiamine Deficiency ; Wernicke Encephalopathy

OBJECTIVETo make through introduction of Wernicke's encephalopathy (WE) following hematopoietic stem cell transplantation (HSCT) in terms of clinical characteristics, diagnostic process and treatment.

METHODSThe clinical charactaristics, diagnostic and therapeutic process and prognostic follow-up in 4 patients diagnosed of WE following HSCT between January 2016 to January 2017 at Department of Hematology, Chinese Aerospace Center Hospital were retrospectively analyzed.

RESULTSFour patients included 2 ALL and 2 AML, and 3 males and 1 female, their age ranged from 8 to 20 years old. 4 patients accouted for about 3% of all petients who received HSCT at that time. Typical triad syndrome consisting of ocular motility disorders, ataxia, global confusion was seen in only 1 patient. However, confusion and heterophthongia as onset of this complication were seen in all patients. Cerebral computed tomograph scan was universally unremarkable and useless. Cerebral MRI scan disclosed that typical involvement including thalamus, fourth ventricle, third ventricle, middle cerebral aqueduct was seen in 3, while untypical site including mamillary body was in the remaining 1 patient. All received vitamin B supplement therapy by intramuscular injection at a dose of 100 mg each day. Initial response was observed at 2, unknown, 3, 4 days after treatment and all obtained complete remission within 2 weeks without any event of relapse after median follow-up period of 8 (7-12) months.

CONCLUSIONAny recipient of HSCT with clinical signs or symptoms of central nervous system should receive vitamin B supplementary therapy immediately to decrease risk of mortality of WE even if the diagnosis of WE is uncertain.

Adolescent ; Child ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Magnetic Resonance Imaging ; Male ; Retrospective Studies ; Thiamine ; Wernicke Encephalopathy ; Young Adult

No abstract available.
Marchiafava-Bignami Disease ; Wernicke Encephalopathy

No abstract available.
Drug Therapy ; Lymphoma* ; Wernicke Encephalopathy*

Wernicke encephalopathy is usually accompanied with peripheral neuropathy, known as dry beriberi. In contrast, wet beriberi presenting as cardiovascular symptoms rarely occurs. The acute type of wet beriberi can be fatal, if untreated quickly. It is reported that the cerebellar vermis has a role of the coordination and control of cardiovascular and autonomic reflex activities. We report a 58-year-old man showing acute wet beriberi in Wernicke encephalopathy with cerebellar vermis lesion.
Beriberi* ; Cerebellar Vermis ; Humans ; Middle Aged ; Peripheral Nervous System Diseases ; Reflex ; Wernicke Encephalopathy*

No abstract available.
Humans ; Paralysis* ; Wernicke Encephalopathy*

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.
Ataxia ; Atrophy ; Brain ; Constipation ; Diet ; Enema ; Hand ; Humans ; Hypotension, Orthostatic ; Hypothalamus ; Hypothermia* ; Magnetic Resonance Imaging ; Mammillary Bodies ; Middle Aged ; Nervous System Diseases ; Recurrence ; Skin ; Wernicke Encephalopathy*