Extrapontine myelinolysis in a pediatric case of diabetic ketoacidosis and cerebral edema

Central pontine and extrapontine myelinolysis are characterized by symmetric demyelination following rapid shifts in serum osmolality, although in extrapontine myelinolysis, demyelination is confined to the supratentorial compartment. We present a case of extrapontine myelinolysis in a 17-year-old female that occurred in the setting of diabetic ketoacidosis, cerebral edema, mannitol therapy, and meningitis. The rate of correction of this patient's glucose and electrolyte levels was within well-accepted limits. Extrapontine myelinolysis is rare in pediatric patients: there are only 12 reports of extrapontine myelinolysis in children under age 20 years and no pediatric cases of extrapontine myelinolysis or central pontine myelinolysis associated with diabetic ketoacidosis. We review the published cases of extrapontine myelinolysis and examine the underlying etiologies and electrolyte disturbances that characterize these cases. This case expands the list of conditions in which extrapontine myelinolysis occurs to include pediatric patients with complicated diabetic ketoacidosis, emphasizing the importance of sudden osmolar shifts in the genesis of this disorder.     

Extrapontine myelinolysis after surgical removal of a pituitary tumour

Salvesen R. Extrapontine myelinolysis after surgical removal of a pituitary tumour.
Acta Neurol Scand 1998: 98: 213–215. Munksgaard 1998.
Extrapontine myelinolysis is a rare and serious disorder characterized by patches of demyelination in certain areas of the brain. Common sites of involvement are the basal ganglia and the thalamus. The patient most often presents with behavioural abnormalities, including mutism, and extrapyramidal symptoms and signs. The diagnosis is established by magnetic resonance imaging. It usually carries a grave prognosis. The pathogenesis seems to be related to profound hyponatremia that is corrected with infusions of saline. We report a case that underwent surgical removal of a pituitary tumour and subsequently was treated with desmopressin. Probably due to an overdose of this antidiuretic hormone, she became obtunded and was found to have profound hyponatremia. This was corrected with infusions of saline, mostly isotonic. She later developed mental disorientation and mutism, and magnetic resonance imaging demonstrated myelinolysis in the basal ganglia. In the course of several months, she has made some recovery, though still demonstrating some memory deficit.     

脑桥中央髓鞘溶解症一例

患者男,30岁,因反复发热1月,四肢无力4 d于2008年11月7日入住南海人民医院神经内科.患者入院1月前无明显诱因出现畏寒发热,最高体温38.5℃,在外院予退热、抗感染等处理,仍反复发热.入我院前4d,患者出现四肢无力,伴头痛、言语不清,并进行性加重,不能站立.既往体健,无酗病史.查:体温36.5℃,脉搏60次/min.呼吸18次/min,血压90/60 mm Hg,心肺腹无明显异常.     

Benign myoclonus of early infancy: a case report

The author reports a male infant with benign myoclonus of early infancy (BMEI). Series of spasms were first seen at the age of 6 months, then occurred from once to three times a day while he was awake, but never during sleep. The first examination at the age of 8 months revealed a normally developed infant with no neurological abnormality. Both interictal- and ictal-electroencephalograms (EEG) were normal. During three months of follow-up observation with no medication, the spasms completely disappeared. He was subsequently observed up to 2 years and 0 month of age and showed normal psychomotor development, normal EEG with no epileptic discharges, and absence of any types of seizures. The spasms associated with BMEI have been considered to be a non-epileptic phenomenon. They are benign and disappear spontaneously without any complication. Therefore, it is important to differentiate BMEI from various epileptic syndromes, especially West syndrome, to avoid unnecessary administration of antiepileptic drugs.     

Melanotic neuroectodermal tumor of infancy: a case report

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare pigmented tumor generally occurring in the head and neck region in children 12 months of age or younger. Approximately 380 instances of this tumor are reported in the medical literature. We presented a case of MNTI that occurred in the left temporal bone of a 2-month old female infant. And, the clinical assessment, histologic diagnosis, and management is reviewed.     

Intraventricular meningioma: case report in infancy

Meningiomas are infrequent tumours in infancy but when they appear in this age group, the intraventricular location is more frequent than in adults. We report the case of a 5-year-old girl with intracranial hypertension and a VI nerve palsy. Computed tomography and magnetic resonance imaging of the brain revealed a tumoral lesion involving the right lateral ventricle with intense enhancement after contrast administration. It was completely resected through a mean temporal circunvolution route. Histological diagnosis was of atypical meningioma.     

脑桥中央合并脑桥外髓鞘溶解症一例

脑桥中央髓鞘溶解症(central pontine myelinolysis,CPM)是原因不明的以脑桥基底部对称性脱髓鞘为病理特征的综合征,常发生在快速纠正低钠血症之后.病灶若出现在脑内的其他部位,如双侧豆状核、尾状核、丘脑和皮层下白质等,则称为脑桥外髓鞘溶解症(extrapontine myelinolysis,EPM),其临床相对少见.现将收集的一例CPM合并EPM报道如下.     

Extrapontine myelinolysis with involvement of the hippocampus in three children with severe hypernatremia

Central pontine myelinolysis is a disorder of unknown etiology linked to overly aggressive correction of hyponatremia. In addition to the typical location of demyelination with preservation of neurons and axon cylinders in the basis pontis, similar lesions have been described in extrapontine locations. Central pontine myelinolysis and extrapontine myelinolysis usually occur together, and are identified at autopsy rather than in life because symptoms of extrapontine myelinolysis are often masked in the critically ill patient. Central pontine myelinolysis is described in children, usually in the clinical setting of hyponatremic dehydration. Extrapontine myelinolysis has not been described in children previously. We report three children with severe hypernatremia and extrapontine myelinolysis involving various combinations of thalamus, basal ganglia, external and extreme capsules, and cerebellar vermis. All three had additional involvement of the hippocampus seen on T2-weighted magnetic resonance imaging. None of the three had detectable pontine lesions. Clinical features of the three cases were dehydration in a 28-month-old girl, respiratory syncytial virus bronchiolitis in a 14-month-old girl, and acute respiratory failure due to anaphylaxis after consumption of walnuts in a 3-year-old boy. Peak sodium values in each child were 195, 168, and 177 mmol/L, respectively; each received aggressive treatment for hypernatremia. We believe this to be the first report of extrapontine myelinolysis in children, the first report of extrapontine myelinolysis without central pontine myelinolysis in children, and the first report in children of hippocampal formation involvement. The pathogenesis of the central and extrapontine myelinolysis complex in children is more complicated than previously believed, and might differ significantly from that of adults.     

8例脑桥中央髓鞘溶解症分析

目的 研究脑桥中央髓溶解症（CPM）的临床特点和影像学改变.方法 对2000年1月～2009年12月我院诊治的8例CPM患者的临床特点及影像学进行回顾性分析.结果 CPM的病因主要与慢性酒精中毒、营养不良、快速纠正低钠血症等有关,临床以假性延髓性麻痹和四肢痉挛性瘫痪为典型表现.MRI扫描可见脑桥中央髓鞘脱失灶.结论 CPM是一种相对罕见的脱髓鞘疾病,临床症状及体征是诊断CPM的基础,头部MRI是诊断该病的重要手段.     

1例缓慢补钠致脑桥中心／脑桥外髓鞘溶解症分析

目的 提高对危重患者救治过程中发生脑桥中央髓鞘溶解症（CPM）/脑桥外髓鞘溶解症（EPM）的认识.方法 对1例危重患者救治过程中发生CPM/EPM的临床资料、辅助检查及相关文献进行分析.结果 对营养不良、严重感染伴有低钠血症的患者,每日纠正血钠10mmol/L亦可发生渗透性髓鞘溶解症,核磁检查显示病灶更清晰.结论 慢性低钠血症患者为避免出现CPM/EPM,每日血钠纠正应＜10mmol/L.     

MRI of cerebral vein thrombosis in infancy: a case report

We diagnosed primary idiopathic cerebral vein thrombosis in an infant by MRI. The relative noninvasiveness of the scan, ease of imaging in multiple planes, and good image resolution suggest that the scan may be useful in this disorder.     

Benign paroxysmal torticollis in infancy: case report

Since Snyder reported first twelve cases of benign paroxysmal torticollis in infancy (BPT) in 1969, about 30 cases have been reported mainly in the North America and Europe, but not in Japan. The authors experienced such a case of one-year-old boy. The attack occurred at the age of 2 months for the first time and continued to repeat every 2 weeks periodically. The head tiltings were always noticed when he woke up in the morning and lasted for about 5 to 6 days, but they disappeared during sleep. He tilted his head to the lateral side more often to the left than to the right. There were associated symptoms and signs including irritability, vomiting, gait disturbance, and abnormal truncal posture during the attacks. He did not demonstrate any neurological abnormalities between the attacks. The study of cerebrospinal fluid, caloric test, computed tomography, electroencephalography and brainstem auditory evoked responses were also normal. The frequency of the attacks gradually decreased after the age of 2 years and disappeared at the age of 3 years, and he has not complained of severe headache or vertigo up to 6 years of age. This case is reported to emphasize that BPT has to be included in the differential diagnosis of acquired torticollis. And it is required to accumulate such cases to elucidate its etiological factors.     

Pathogenesis and prevention of central pontine myelinolysis: A case report

Central pontine myelinolysis (CPM) is a demyelinating disease producing serious or even fatal illness. The cause of the disease has been primarily attributed to alcoholism. Recently hyponatremia and its rapid correction have been related to CPM. We describe an alcoholic patient who developed the disease. The case has given us the opportunity of reflecting on CPM pathogenesis and management.

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Sommario La mielinolisi centrale del ponte è una malattia demielinizzante che può anche essere fatale. La sua causa è stata, in principio, riconosciuta nell;alcolismo. Recentemente l’iponatremia e la sua rapida correzione sono state messe in rapporto alle mielinolisi. In questa nota viene descritto un paziente alcolista cronico affetto da una forma lieve di mielinolisi centrale del ponte. L’osservazione di questo caso ha suggerito interessanti considerazioni patogenetiche e terapeutiche più generali.