Extradural arachnoid cysts in children

Background Extradural arachnoid cysts in the spine are uncommon causes of spinal cord compression in the pediatric population that are thought to arise from congenital defects in the dura mater. Most reports describe such cysts as communicating with the intrathecal subarachnoid space through a small defect in the dura. Excision of the cyst with obliteration of the communicating dural defect is the mainstay of treatment in symptomatic patients. Solitary extradural arachnoid cysts have been reported in several studies, but multiple extradural arachnoid cysts are very rarely reported in children. Materials and methods The authors report a case of multiple extradural spinal arachnoid cysts in a 14-year-old boy who presented progressive lower extremity weakness, myelopathy, and severe gait ataxia. Magnetic resonance (MR) of the spine demonstrated four extradural arachnoid cysts extending from T-1 to T-9. The patient underwent a thoracic laminoplasty for en bloc resection of the spinal extradural arachnoid cysts. Postoperatively, the patient’s motor strength and ambulation improved immediately. Conclusions Multiple spinal extradural arachnoid cysts are rarely reported in the literature. Excision of the cysts at the spinal cord level leads to a favorable outcome.     

An autopsy case of ornithine transcarbamylase deficiency

We present an autopsy case of ornithine transcarbamylase (OTC) deficiency with grumose degeneration in the dentate nucleus of the cerebellum. The patient had intractable neonatal convulsions and hyperammonemia from the 3rd day after birth. Diagnosis of OTC deficiency was made based on null activity of the enzyme and four-base deletions in exon 9 of the OTC gene. Death was due to sepsis as well as disseminated intravascular coagulation at 1 year and 2 months of age. Neuropathology showed multiple cystic changes and ulegyria in the bilateral frontal and parietal lobes. Multiple cysts were associated with the region, which was infiltrated with macrophages surrounded by astroglia showing palisading pattern. Ferrugination was marked in the thalamus and severe neuronal loss with astrogliotic change in the CA1-2 area of the hippocampus. Grumose degeneration was noted in the dentate nucleus of the cerebellum. This is the first report of grumose degeneration in OTC deficiency.     

Spinal meningeal malformations in children (without meningoceles or meningomyeloceles)

Multiple meningeal malformations are described: anterior or lateral meningoceles, extradural meningeal cysts, and intradural arachnoid cysts. All diverticulae appear to be extensions of the subarachnoid space, producing symptoms early or later. It is impossible to unify all these lesions because they cause multiple pathological conditions, depending upon the anatomical form or level, other systemic malformations, spinal abnormalities, or associated familial diseases. Surgical treatment requires complete evaluation of each anatomical aspect before procedure.     

Rathke''s cleft cyst

A 29 year old female with a chiasmal syndrome secondary to a Rathke's cleft cyst is described. The histology and pathogenesis of these rare cysts are discussed, contrasting them with other hypophysial cysts.     

Evaluation of the enzyme-linked immunoelectrotransfer blot assay for diagnosis of neurocysticercosis in children

Neurocysticercosis is a common problem in developing countries, and it causes neurologic disorders in children. Immunodiagnosis with Taenia solium glycoproteins as an antigen has been validated in adults but not in children. The aim of this work was to evaluate a Taenia solium glycoproteins-based enzyme-linked immunoelectrotransfer blot assay in children with neurocysticercosis. Twenty-five confirmed cases of neurocysticercosis and 50 healthy children from the same community were included. The test had a sensitivity of 72% and a specificity of 96%. Sensitivity was higher (100%) in cases with multiple cysts and in multiple sites. Sensitivity was higher when cysts were in parenchyma (86%) than when they were in the subarachnoid space. The most frequently recognized proteins were 24, 39 to 42, and 50 kDa. Diagnosis was more efficient in serum than in cerebrospinal fluid. Western blot is a reliable method for serologic diagnosis of neurocysticercosis in children. Multiple cysts and infections in multiple sites elicited a stronger immune response.     

Should we resect all multiple intraspinal extradural arachnoid cysts? A case report

Multiple spinal extradural arachnoid cysts (SEACs) are relatively rare lesions, which may cause symptoms due to spinal cord compression. Radical resection of multiple SEACs during one-stage surgery is the primary treatment for the rare lesion, because of its effective and safe operation. Here, we present a 13-year-old female, who presented with a 3-month history of progressive weakness in the right leg. For the first time, we reported partial resection of multiple SEACs without treating the other parts, can also lead to a favorable outcome. No change in the cysts and spinal deformity were observed during a follow-up period of 5 years.     

Multiple infected cerebral hydatid cysts.

We report an unusual patient with multiple infected cerebral hydatid cysts. A 20-year-old man presented with a 2-month history of headache and progressive left-sided hempiparesis. A cerebral CT scan showed a large and heterogeneous parieto-occipital lesion. During surgery an infected hydatid cyst was discovered with multiple daughter vesicles. Post-operatively the patient was treated with albendazol, cefotaxime and metronidazole. The clinical course was good with total recovery of the hemiparesis. A follow-up CT scan showed persistence of some small deep-seated cysts. Multiple infected cerebral hydatid cyst is uncommon and can be confused with other cystic brain lesions. The aim of surgery is to remove the cyst unruptured and this should be followed by antihelminthic and antibiotic treatment in order to avoid recurrences.     

Multicystic acoustic neurinoma: report of two cases.

A report of two unusual and remarkably similar cases of multicystic acoustic neurinoma in 46 and 60 year old patients is presented. The clinical presentation mimicked a regular acoustic neurinoma. The tumours in each case were similar in their radiological appearance. The entire tumour had a honeycomb appearance with well defined and multiple cysts. Each cyst contained xanthochromic fluid. Like in any other cystic acoustic neurinoma, the presence of multiple cysts made internal decompression of the tumour relatively easy but the dissection of the tumour from the adjoining cranial nerves was difficult. The pathogenesis of multiple cysts is analyzed and the literature on this subject is briefly reviewed.     

Brain stem glioblastoma with multiple large cyst formation and leptomeningeal dissemination in a 4-year-old girl

The authors report a 4-year-old girl who developed brain stem glioblastoma. Meningeal irritation was present at onset. Magnetic resonance imaging revealed intracranial and intraspinal leptomeningeal dissemination, which progressed faster than the original tumor. Multiple large cysts developed at the interhemispheric and prepontine cisterns, resulting in progressive obstructive hydrocephalus. The patient survived only 5 months after presentation. Histology was verified by autopsy.     

Multiple cerebral hydatid cysts. Report of a case with 24 pieces

A case of 19 years old female patient with 24 large primary cerebral hydatid cysts, in different localisations is presented. Cysts were removed unruptured by five separate operations in 6 months. The patient was observed to be in good health 1 year after the last operation. This case is the first case presented that contains such high number of cysts to our knowledge. The importance of MRI examination in localizing the cysts and designing the operative plan is emphasized. Interestingly, albendazole, which is an antiparasitic drug, was reported to be ineffective by infectious disease department of our hospital, because of large diameter of the cysts, in this case.     

Leukoencephalopathy with Anterior Temporal Cysts Due to Congenital CMV Infection Diagnosed Retrospectively

Leukoencephalopathy with subcortical cysts has been described in a variety of conditions. However, few reports have highlighted congenital CMV as a cause of this imaging finding. We report a 1‐year‐old girl with developmental delay and sensorineural hearing loss whose MRI brain showed abnormal white matter and temporal cysts. Congenital CMV infection was diagnosed retrospectively by examination of dried blood spot from the newborn screening card.     

Growth of multiple hydatid cysts evaluated by computed tomography

A case of multiple hydatid cysts, of embolic origin from a cyst of the left atrium, is discussed. The patient was first admitted to hospital with an intracerebral haemorrhage and was treated conservatively. There was not any cystic or mass lesion other than intracerebral haemorrhage on his computed tomography (CT) scans. In the follow-up period, the CT scans after 14 months showed multiple hydatid cysts, with the largest about 6 cm in diameter, which suggested a growth rate of 5 cm per year.     

Multiseptated arachnoid cyst treated with fenestration after valvular insufficiency in an adult.

Treatment of symptomatic arachnoid cysts is based on two procedures: cyst fenestration versus derivation of CSF. Multiseptated cysts represent a very special group. We present the case of a 75 year old woman with a symptomatic multiseptated arachnoid cyst, developing subacute bleeding in one of the cavities. Final diagnosis was obtained after MRI. CSF derivation became insufficient with clear improvement after fenestration and communication to subarachnoid space.     

Intraepithelial carcinoma arising in an endodermal cyst of the posterior fossa

Endodermal cysts of the central neuraxis are benign, non‐neoplastic epithelium‐lined cysts arising from endodermal tissue that have been displaced early in fetal life. Intracranial endodermal cysts are rare and usually located in the posterior fossa. The present study involves a 36‐year‐old man with a typical epithelial cyst in the posterior fossa. Microscopically, the cyst has a simple columnar epithelium with mucus‐producing cells, containing an area composed of dysplastic epithelium with evidence of an intraepithelial carcinoma. The atypical cells have a high proliferative fraction demonstrated by Ki‐67 immunostain. Based on these findings, the authors view this case as evidence of a malignant transformation of a classic endodermal cyst. The clinicopathologic features and a review of the literature are discussed.     

Cauda equina syndrome caused by Tarlov's cysts--case report

Perineural Tarlov cysts located on lumbo-sacral roots can be a cause of cauda equina syndrome. OBJECTIVES: 1) To draw attention to the fact that multiple Tarlov lumbo-sacral perineural cysts can produce serious movement disturbances. 2) To document the usefulness of the magnetic resonance imaging in noninvasive diagnosis of perineural cysts. CASE DESCRIPTION: A male patient, 80 years of age, suffered from progressive weakness of lower limbs, which caused an increasing drop of the feet. The disease began in August 2000, following a long journey by train. The patient additionally complained of urinary incontinence as result of sneezing, coughing or fast walking. The urologist did not find prostatic gland hypertrophy. An examination by the internist revealed atheromatous myocardiopathy in circulation failure stage. Magnetic resonance imaging showed multiple perineural cysts up to 15 mm in diameter on lumbo-sacral roots. This clinical picture, supported by the magnetic resonance imaging allowed to recognize cauda equina syndrome caused by Tarlov lumbo-sacral perineural cysts. DISCUSSION: This case is a reminder, that part of perineural cysts, particularly multiple, can be a cause of nerve roots injury, and their lumbo-sacral location can produce cauda equina syndrome. As reported by Zarski and Leo, Tarlov cysts were cause of 7.3% of pain syndrome cases 2 patients in the study group showed lower limb claudication. Magnetic resonance imaging of patients with back pain, performed by Paulsen, Call and Murtagh, revealed that Tarlov cysts occurred in 4.6% of patients, but only 1% had the symptoms connected with the presence of those cysts. In available Polish literature no report has been found referring to fixed cauda equina syndrome which was caused by multiple cysts revealed through the magnetic resonance imaging of spinal canal. Only Zarski and Leo, discussing the correlation between the clinical and radicographic picture, described transient cauda equina syndrome in two patients who, beside Tarlov cysts, were also found to have intervertebral lumbosacral disc herniation. Tarlov was the first to describe well documented cauda equina syndromes caused by cysts on the lumbo-sacral roots. It is necessary to emphasize the established role of magnetic resonance of spinal canal in the diagnosis of perineural cysts on the lumbo-sacral roots as well as other anatomical anomalies of cerebrospinal fluid spaces. Despite the fact that cauda equina syndrome in the case reported here was a serious complication of multiple Tarlov cysts in the lumbo-sacral region, a surgical treatment was not undertaken; in such cases this treatment should be the chosen procedure. CONCLUSION: Multiple perineural Tarlov cysts in lumbo-sacral region, without disc herniation or other cause of vertebral canal stenosis, can produce cauda equina syndrome.     

Neuroepithelial cysts presenting with movement disorders: two cases

BACKGROUND: The authors present two cases of movement disorders caused by neuroepithelial cysts and highlight their management. Neuroepithelial cysts are ependymal or epithelial lined fluid collections of unknown etiology within the central nervous system parenchyma with no obvious ventricular or subarachnoid connection. Most cysts are asymptomatic, however, some present with seizures, mass effect, or rarely with movement disorders. CASE REPORTS: The first patient, a 27-year-old female, presented with progressive weakness, dystonic posturing, tremor, ballismus and choreoathetotic movements of her right upper extremity. Her symptoms improved after stereotactic drainage of a neuroepithelial cyst in her basal ganglia but recurred within a year. The second case, a 56-year-old female, presented with diplopia, nystagmus, gait imbalance and hemiparesis. Her symptoms improved after stereotactic drainage of a midbrain neuroepithelial cyst. The cyst reaccumulated over the next few years and she became symptomatic with left arm tremor and facial weakness. Aspiration was again performed with symptomatic improvement for nine months. Her tremor recurred and a cyst access device was placed stereotactically. She improved and has remained stable for over a year. CONCLUSION: Simple stereotactic drainage of neuroepithelial cysts has a high recurrence rate. The authors recommend considering placement of a drainage device to facilitate aspiration of the cyst fluid during follow-up, if needed.     

Cestode parasitic infestation: intracranial and spinal hydatid disease--a clinicopathological study of 29 cases from South India

In developing countries hydatidosis is both a medical and economic problem related to environmental hygiene and healthy veterinary practice. This cestode parasitic infestation, uncommonly involving the nervous system, presents with varied clinical manifestation, at times causing diagnostic dilemmas. Multiple intracranial and spinal hydatidosis is rare. A series of 29 histologically confirmed cases of hydatidosis of neuraxis (21 intracranial and 8 spinal) from South India are presented. Among the 21 cases of intracranial hydatidosis, 12 cases were in pediatric age, while only 1 spinal lesion was noted in a 5-year-old child. The clinical presentation of intracranial lesions was predominantly that of raised intracranial pressure and visual symptoms, while spinal hydatidosis manifested with severe back pain, weakness and sphincter disturbances. The cranial cysts were usually single and uniloculated (12 cases), multiple in 7 and single but multiloculated in 2. In spinal hydatidosis, the cysts are usually multiple and extradural, rare ones being intramedullary and intradural. Based on clinical features and imaging, the differential diagnosis for intracranial lesions were cystic tumors and arachnoid cyst while metastasis and tuberculosis were considered in cases of spinal hydatidosis because of vertebral bony involvement. The majority of the cysts could be surgically resected totally and some were aspirated under control suction and resected. None of the cases had anaphylactic reaction, with no significant post-operative morbidity and no mortality. One intracranial and 2 spinal lesions caused by fertile cysts recurred to undergo repeated surgery.     

成人症状性骶管囊肿的外科治疗

目的 探讨成人症状性骶管囊肿的治疗方法及疗效。方法 2013年1月至2015年4月收治症状性骶管囊肿32例,均在术中监护下手术,采取囊壁大部分切除+漏口缩窄+自体脂肪瓣填塞术17例,囊壁切除+交通孔缝扎+自体脂肪瓣填塞术8例,囊壁部分切除+神经袖套重建+自体脂肪瓣填塞术7例。采用Oswestry功能障碍指数及改良腰椎日本骨科协会（JOA）评分评估手术前后功能状态及疼痛程度。结果 术后随访12~36个月,平均22个月,无复发;29例症状改善,功能恢复良好,术后1年Oswestry功能障碍指数及改良腰椎JOA评分均较术前明显改善（P<0.01）。结论 依据骶管囊肿的分型,采取相应手术,可缓解患者疼痛及改善患者生活质量。     

磁共振动态脑脊液流速测定技术在蛛网膜囊肿诊治中的应用

目的 探讨MRI动态脑脊液流速测定技术(MRI电影技术)对蛛网膜囊肿诊断及治疗的价值.方法 用普通MRI、CT对17例不同部位的蛛网膜囊肿患者进行平扫+增强,同时对其中的14例患者,应用MRI电影技术分别对蛛网膜囊肿内液体与蛛网膜下腔内脑脊液测速,并对所测得的结果进行统计学分析.结果 与沟通性蛛网膜囊肿不同,非沟通性蛛网膜囊肿内液体流速与蛛网膜下腔内脑脊液流速差异有统计学意义(P＜0.05),并于术中证实.结论 与普通MRI和CT对比,MRI电影技术可以无创性诊断蛛网膜囊肿是否与外界沟通,给手术治疗提供证据.     

Parkinsonism with multiple cysts in the bilateral striata

The present paper reports on a 68‐year‐old man with a 10‐year history of parkinsonism who developed hallucinations and delusions after admission to an intensive care unit for the treatment of organophosphate intoxication. His initial diagnosis was delirium. On the basis of brain computed tomography findings and clinical symptoms, we diagnosed drug‐induced psychosis in parkinsonism with multiple cysts in the bilateral striata.