RESUMO
A 41-year old woman was treated for cholera at one of the health centers in Blantyre. Two days after discharge from the treatment unit, she developed weakness of all 4 limbs and difficulties with speech. She was referred to the Queen Elizabeth Central Hospital. A CT scan of the brain showed hypodense lesions in the pons. A diagnosis of central pontine myelinolysis was made. She recovered slowly and was discharged from hospital 17 days after admission.
Assuntos
Cólera , Mielinólise Central da Ponte , Feminino , Humanos , Adulto , Cólera/complicações , Cólera/diagnóstico , Cólera/patologia , Mielinólise Central da Ponte/diagnóstico , Mielinólise Central da Ponte/patologia , Ponte/patologia , Encéfalo , Tomografia Computadorizada por Raios X , Imageamento por Ressonância MagnéticaRESUMO
ABSTRACT: Central pontine myelinolysis is most commonly associated with rapid correction of hyponatremia and has historically been associated with alcoholism. In this case report, 2 deaths with gross findings of central pontine lesions led to the possibility that CPM may have been a potential mechanism of death. Subsequent analysis revealed that these lesions were incidental findings. This case report discusses the importance of appropriate microscopic and immunohistochemical analysis of suspected CPM cases.
Assuntos
Alcoolismo , Hiponatremia , Mielinólise Central da Ponte , Alcoolismo/complicações , Alcoolismo/patologia , Humanos , Hiponatremia/complicações , Hiponatremia/patologia , Imageamento por Ressonância Magnética/efeitos adversos , Mielinólise Central da Ponte/complicações , Mielinólise Central da Ponte/patologia , Ponte/patologiaRESUMO
BACKGROUND: Variable neurological manifestations and imaging findings have been described in children with severe hypernatremia. We aimed to describe the spectrum of neuroimaging changes in infants with severe hypernatremia. METHODS: This retrospective study included infants with severe hypernatremia (serum sodium >160 mEq/L), abnormal neurological examination, and an abnormal magnetic resonance imaging (MRI) of the brain over a period of 2 years in a tertiary care hospital. Relevant clinical data, including the feeding practices, clinical features, complications, and biochemical and radiological parameters, were entered in a structured pro forma. MRI findings were classified as vascular (hemorrhages and cerebral sinus venous thrombosis), osmotic demyelination syndrome (pontine and extrapontine myelinolyses), and white matter changes. RESULTS: The common clinical features in the neonates were poor feeding (n = 4) and decreased urine output (n = 4); the older infants presented with gastrointestinal losses (n = 5). All cases had dehydration with encephalopathy. The patterns of radiological injury were vascular (hemorrhages, n = 5 and venous thrombosis, n = 3), osmotic demyelination (n = 8), and white matter changes (n = 7). Coagulopathy was correlated with the vascular complications (r = 0.8, p < 0.0001); the degree of dehydration was correlated with the venous thrombosis (r = 0.7, p < 0.04) and acute kidney injury (r = 0.8, p < 0.001). Neurological sequelae were seen in four cases and correlated with hypernatremia (r = 0.6, p = 0.03) and hyperosmolarity (r = 0.6, p = 0.03). CONCLUSION: Characteristic neuroimaging findings are vascular changes in the form of venous thrombosis and hemorrhages, osmotic demyelination and white matter tract injury, and/or mostly combinations of these findings. Severe hypernatremia and resulting hyperosmolarity frequently cause neurological sequelae in neonates and infants.
Assuntos
Hipernatremia , Mielinólise Central da Ponte , Criança , Humanos , Hipernatremia/complicações , Hipernatremia/etiologia , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética/métodos , Mielinólise Central da Ponte/diagnóstico , Mielinólise Central da Ponte/etiologia , Mielinólise Central da Ponte/patologia , Neuroimagem/efeitos adversos , Estudos RetrospectivosRESUMO
Demyelinated lesions in human pons observed after osmotic shifts in serum have been referred to as central pontine myelinolysis (CPM). Astrocytic damage, which is prominent in neuroinflammatory diseases like neuromyelitis optica (NMO) and multiple sclerosis (MS), is considered the primary event during formation of CPM lesions. Although more data on the effects of astrocyte-derived factors on oligodendrocyte precursor cells (OPCs) and remyelination are emerging, still little is known about remyelination of lesions with primary astrocytic loss. In autopsy tissue from patients with CPM as well as in an experimental model, we were able to characterize OPC activation and differentiation. Injections of the thymidine-analogue BrdU traced the maturation of OPCs activated in early astrocyte-depleted lesions. We observed rapid activation of the parenchymal NG2+ OPC reservoir in experimental astrocyte-depleted demyelinated lesions, leading to extensive OPC proliferation. One week after lesion initiation, most parenchyma-derived OPCs expressed breast carcinoma amplified sequence-1 (BCAS1), indicating the transition into a pre-myelinating state. Cells derived from this early parenchymal response often presented a dysfunctional morphology with condensed cytoplasm and few extending processes, and were only sparsely detected among myelin-producing or mature oligodendrocytes. Correspondingly, early stages of human CPM lesions also showed reduced astrocyte numbers and non-myelinating BCAS1+ oligodendrocytes with dysfunctional morphology. In the rat model, neural stem cells (NSCs) located in the subventricular zone (SVZ) were activated while the lesion was already partially repopulated with OPCs, giving rise to nestin+ progenitors that generated oligodendroglial lineage cells in the lesion, which was successively repopulated with astrocytes and remyelinated. These nestin+ stem cell-derived progenitors were absent in human CPM cases, which may have contributed to the inefficient lesion repair. The present study points to the importance of astrocyte-oligodendrocyte interactions for remyelination, highlighting the necessity to further determine the impact of astrocyte dysfunction on remyelination inefficiency in demyelinating disorders including MS.
Assuntos
Astrócitos/fisiologia , Diferenciação Celular , Mielinólise Central da Ponte/patologia , Células Precursoras de Oligodendrócitos/fisiologia , Oligodendroglia/fisiologia , Adulto , Idoso , Animais , Antidiuréticos , Astrócitos/patologia , Linhagem da Célula , Desamino Arginina Vasopressina , Doenças Desmielinizantes/metabolismo , Doenças Desmielinizantes/patologia , Modelos Animais de Doenças , Feminino , Humanos , Ventrículos Laterais/citologia , Ventrículos Laterais/metabolismo , Masculino , Pessoa de Meia-Idade , Bainha de Mielina , Mielinólise Central da Ponte/induzido quimicamente , Mielinólise Central da Ponte/metabolismo , Proteínas de Neoplasias/metabolismo , Nestina/metabolismo , Células-Tronco Neurais , Células Precursoras de Oligodendrócitos/metabolismo , Oligodendroglia/metabolismo , Ratos , Cloreto de SódioRESUMO
OBJECTIVE: This study describes the MRI changes associated with pontine and extrapontine myelinolysis secondary to rapid correction of hyponatremia in dogs. The authors discuss the relevance of the results for theories of pathogenesis and for diagnosis of patients. RESULTS: MRI changes associated with pontine and extrapontine myelinolysis first occur on diffusion-weighted imaging. As a generalization, gadolinium enhancement, flair image change and T2 weighted image abnormality appear sequentially.
Assuntos
Doenças do Cão/diagnóstico por imagem , Hiponatremia/complicações , Imageamento por Ressonância Magnética/métodos , Mielinólise Central da Ponte/diagnóstico por imagem , Animais , Doenças do Cão/etiologia , Doenças do Cão/patologia , Cães , Mielinólise Central da Ponte/etiologia , Mielinólise Central da Ponte/patologiaRESUMO
BACKGROUND: Here, we report a case of central pontine demyelinization in a type-2 diabetes patient with hyperglycemia after a binge-eating attack in the absence of a relevant hyponatremia. CASE PRESENTATION: A 55-year-old, male type-2 diabetic patient with liver cirrhosis stage Child-Pugh B was admitted due to dysmetria of his right arm, gait disturbance, dizziness, vertigo, and polyuria, polydipsia after a binge-eating attack of sweets (a whole fruit cake and 2 Liters of soft drinks). A recently initiated insulin therapy had been discontinued for 8 months. A serum glucose measurement obtained 5 days prior to hospitalisation was 38.5 mmol/l (694 mg/dl). The patient graved for sweets since stopping alcohol consumption 8 months earlier. On admission, venous-blood glucose was 29.1 mmol/l (523.8 mg/dl), glycated hemoglobin was 168.0 mmol/mol or 17.6%. No supplementation of sodium chloride was reported. Laboratory exams revealed an elevated serum ammonia level (127.1 µmol/l), rendering a hepatic encephalopathy very likely. After initiation of insulin therapy, capillary glucose normalized, and serum sodium rose from 133 on admission to 144 mmol/l during the hospital stay. In retrospect, the mild hyponatremia on admission was classified as pseudohyponatremia due to hyperglycemia. The patient had an insulin resistance (HOMA-IR 7.8 (normal range < 2.5)). A T2-weighted magnetic resonance imaging (MRI) of the head and a cranial computed tomography scan were obtained demonstrating a symmetric central pontine demyelinization. After 26 days in hospital, the patient was discharged with an inkretin-mimetic therapy (dulaglutide SC, 1.5 mg/week) and an intensified conventional insulin therapy (insulin aspart: 14 units/d in euglycemia, insulin glargin 20 units/d). CONCLUSIONS: Central pontine and/or cerebellar myelinolysis can be caused by sudden, severe, and sustained hyperglycemia, especially when another risk factor (in this case, liver cirrhosis) is present. Functional neurological deficits in the context of hyperglycemia should prompt for the consideration of this differential diagnosis in all diabetes patients.
Assuntos
Bulimia/complicações , Diabetes Mellitus Tipo 2/fisiopatologia , Hiperglicemia/etiologia , Mielinólise Central da Ponte/etiologia , Humanos , Hiperglicemia/patologia , Masculino , Pessoa de Meia-Idade , Mielinólise Central da Ponte/patologia , PrognósticoRESUMO
Central pontine myelinolysis (CPM) is a neurological demyelinating disease of the pons. Although usually associated with rapid correction of hyponatremia, CPM may occur despite normonatremia, is often associated with chronic alcoholism and may be asymptomatic. Histological confirmation of asymptomatic CPM is rare. We describe an unusual post-mortem case of extensive but asymptomatic CPM in a chronic alcoholic patient with normonatremia. The affected part of the pons contained thinly myelinated axons with appearances supporting remyelination. We suggest that remyelination may account for the subclinical nature of this patient's CPM.
Assuntos
Alcoolismo/complicações , Mielinólise Central da Ponte/patologia , Ponte/patologia , Remielinização , Humanos , Masculino , Pessoa de Meia-IdadeAssuntos
Falência Hepática/patologia , Mielinólise Central da Ponte/patologia , Miopatias Congênitas Estruturais/patologia , Evolução Fatal , Humanos , Falência Hepática/complicações , Masculino , Pessoa de Meia-Idade , Mielinólise Central da Ponte/complicações , Miopatias Congênitas Estruturais/complicaçõesAssuntos
Alcoolismo/complicações , Mielinólise Central da Ponte/patologia , Idoso , Alcoolismo/diagnóstico , Alcoolismo/tratamento farmacológico , Encéfalo/patologia , Humanos , Hiponatremia/diagnóstico , Imageamento por Ressonância Magnética/métodos , Masculino , Mielinólise Central da Ponte/complicações , Mielinólise Central da Ponte/tratamento farmacológico , Tiamina/uso terapêuticoRESUMO
TOPICALITY: Central pontine and extrapontine myelinolysis is a rare and dangerous form of the demyelinating process of undefined origin, the development of which is associated with a very low sodium level in the blood (hypernatremia). OBJECTIVE: To describe a rare case of extrapontine myelinolysis as a complication occurring in the hemorrhagic period of anterior communicating artery aneurysm rupture as well as to demonstrate that this condition can be diagnosed intravitally. CONCLUSION: Clinical vigilance of extrapontine myelinolysis may promote the timely diagnosis and treatment of this disease, which is a potential cause of death. Pulse-therapy with glucocorticoids provides the opportunity to achieve regression of neurological symptoms and to stabilize the patient's condition.
Assuntos
Aneurisma Intracraniano/diagnóstico , Mielinólise Central da Ponte/patologia , Hemorragia Subaracnóidea/diagnóstico , Feminino , Humanos , Aneurisma Intracraniano/complicações , Pessoa de Meia-Idade , Mielinólise Central da Ponte/diagnóstico por imagem , Mielinólise Central da Ponte/etiologia , Hemorragia Subaracnóidea/complicações , Hemorragia Subaracnóidea/etiologiaAssuntos
Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Mielinólise Central da Ponte/terapia , Adulto , Alcoolismo/complicações , Humanos , Angiografia por Ressonância Magnética , Masculino , Mielinólise Central da Ponte/etiologia , Mielinólise Central da Ponte/patologia , SíndromeRESUMO
A 68-year-old female, who suffered from depression, fell down the stairs in her house. She was soon transferred to an accident and emergent ward by an ambulance. Two days later, she was transferred and admitted to our hospital. Next day after admission to our ward, her consciousness level changed for the worse. She developed diabetic ketoacidosis, and was administered insulin during reinfusion. Next day, her diabetic ketoacidosis improved;however, her consciousness level was still poor. Brain magnetic resonance imaging(MRI)showed suspected osmotic myelinolysis lesions in the bilateral cerebellar hemisphere and globus pallidus. After conservative therapy, her lesions almost disappeared in 2 months. We diagnosed the lesions as reversible extrapontine myelinolysis.
Assuntos
Hiperglicemia/complicações , Hiperostose/etiologia , Mielinólise Central da Ponte/etiologia , Idoso , Angiografia Cerebral , Feminino , Humanos , Imageamento por Ressonância Magnética , Mielinólise Central da Ponte/diagnóstico por imagem , Mielinólise Central da Ponte/patologiaAssuntos
Síndrome de Mallory-Weiss/patologia , Mielinólise Central da Ponte/patologia , Ponte/patologia , Alcoolismo/complicações , Alcoolismo/patologia , Autopsia , Biópsia , Causas de Morte , Evolução Fatal , Feminino , Hemorragia/etiologia , Hemorragia/patologia , Humanos , Achados Incidentais , Síndrome de Mallory-Weiss/complicações , Pessoa de Meia-Idade , Mielinólise Central da Ponte/etiologiaRESUMO
Extrapontine myelinolysis is characterized by symmetric demyelination following rapid shifts in serum osmolality in the supratentorial compartment. Extrapontine myelinolysis in children is rare compared to adults. The most common underlying pathophysiology is rapid correction of hyponatremia. Only 2 cases were published after diabetic ketoacidosis without electrolyte imbalance in the English literature. This study presents an unusual and possibly the youngest case of extrapontine myelinolysis that occurred in the setting of diabetic ketoacidosis and complicated cerebral edema without electrolyte imbalance, along with a review of the literature.
Assuntos
Cetoacidose Diabética/complicações , Mielinólise Central da Ponte/complicações , Encéfalo/patologia , Edema Encefálico/sangue , Edema Encefálico/complicações , Edema Encefálico/patologia , Cetoacidose Diabética/sangue , Cetoacidose Diabética/patologia , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Mielinólise Central da Ponte/sangue , Mielinólise Central da Ponte/patologiaRESUMO
A 4-year-old boy presented with a history of tremor for 7 days. He also had recurrent diarrhea for the previous 1 year, and poor weight gain. Magnetic resonance of the brain was suggestive of central pontine myelinolysis. There was no evidence of electrolyte abnormalities. The serum tissue transglutaminase level was markedly elevated, and the duodenal biopsy revealed features of celiac disease. The patient was started on gluten-free diet. The tremor resolved within 3 months. Repeat imaging of the brain done 3 months after starting gluten-free diet showed complete resolution of the lesion. This case highlights the unusual presentation of central pontine myelinosis as tremor in a malnourished child with celiac disease.