Occult intrasacral meningocele: case report and review of the literature

Occult intrasacral meningocele, characterised by the presence of a cyst within the sacral spinal canal, is an unusual congenital lesion. A 26-year-old female presented with a history of low back pain and sensory disturbance in the lower extremities, and urinary incontinence. CT scan of the sacral region showed an asymmetrical widening of the sacral canal and the existence of a thinned bone contour. Magnetic resonance (MR) imaging revealed an intrasacral mass as a low signal intensity area on the T1-weighted image and a high signal intensity area on the T2-weighted image, suggesting a cyst containing cerebrospinal fluid (CSF). The patient underwent sacral laminectomy and posterior exploration showed the presence of sacral erosion and an intrasacral cyst containing CSF devoid of neural contents. Partial surgical excision of the cyst was carried out, but the patient's postoperative course was complicated by a CSF leak due to a small connection that was obliterated in a second operation. Control MR imaging confirmed the removal of the cystic lesion. The clinical, neuroradiological and surgical features of this rare condition are discussed and the importance of a differential diagnosis is stressed.     

Giant intrasacral meningocele: a case report

A peculiar case of intrasacral meningocele and spinal cord tethering is reported. Contents of the intrasacral meningocele and importance of CSF flow analyses with MRI are discussed. Demonstration of CSF flow from the thecal sac to meningocele in the CSF flow MR imaging may be helpful for determining the possibility of meningocele growth. In this report, we have presented the determination of CSF flow as a new surgical indication in this type of cases.     

Occult intrasacral meningocele: case report

Intrasacral meningoceles are rare conditions that can cause symptoms of nerve root compression. We report a case of a 18-year-old female, who presented with urinary incontinence. The neurological and urological evaluation revealed lack of detrusor muscle reflex. This finding supported the hypothesis of spinal disraphism. Magnetic resonance image revealed an intrasacral meningocele. The patient was submitted to surgical excision of the lesion without postoperative complications. In the present paper, the pathogenesis, clinical picture, neuroradiological findings and surgical treatment are discussed.     

Spinal intradural arteriovenous fistula with unusual presentation: case report and literature review

Object  The authors present their experience with a Cervical Spinal intradural arteriovenous fistula (AVF) with unusual clinical presentation in a pediatric patient and review of the international literature on this condition. Methods  A 5-year-old girl was found by her parents in bed screaming and talking inappropriately. Later on, she calmed down and complained of occipital, back and neck pain. She was admitted to the CHEO-ICU for work up and observation. The admission head CT scan showed evidence of SAH. Brain MRI showed blood in the third and fourth ventricles. MRA showed a C2 vascular lesion consistent with spinal cord AVF. Conventional angiogram was done which confirmed the AVF fed by a posterior medullary artery originating from the left vertebral artery. An attempt to embolize the AVF with glue was made but proved to be unsuccessful. On December 4, 2002, direct surgery on the AVF was performed, through a midline posterior approach (C2–C3 laminectomies) and microsurgical technique a complete excision of the intradural extramedullary AVF was achieved. The postoperative course was uneventful and the patient remained neurologically intact. Post-operative angiography confirmed complete excision of the AVF. Conclusion  The rarity of a pediatric spinal AVF presenting with spontaneous Intracranial SAH, makes this case very peculiar. This is the first reported pediatric case of cervical cord AVF with this clinical presentation found in the literature.     

Hematoma in a low-grade medullary astrocytoma: report of an unusual case and literature review

We describe the case – to our knowledge unique – of an 8-year-old boy who presented with acute onset of lower cranial nerve palsy and tetraparesis caused by a hematoma in a dorsal exophytic pilocytic astrocytoma of the medulla oblongata. The boy showed near-complete recovery after neurosurgical management in two stages: first, emergency evacuation of the hematoma with tumor biopsy, and second, complete tumor removal 5 months after the initial event. Intraoperative electrophysiological monitoring techniques for the lower cranial nerves are of value in preserving their functional integrity. Ultrasonography is helpful in assessing the extent of tumor removal. Although the pathological diagnosis of a pilocytic astrocytoma would not justify radiotherapy, local field radiotherapy was added mainly because of the unexpectedly rapid tumor progression during the interval between the two surgical procedures. The literature on brainstem and tumor hematoma in children is reviewed. Received: 26 February 1998     

A case of occult intrasacral meningocele presented with atypical bowel symptoms

Case report We report a case of an occult intrasacral meningocele. An 11-year-old boy presented with atypical bowel symptoms, severe constipation, and stool incontinence. Magnetic resonance (MR) imaging disclosed an intrasacral cyst containing cerebrospinal fluid (CSF). We diagnosed an occult intrasacral meningocele and performed perforation of the cyst and closure of the fistula. The patient was free from constipation and stool incontinence after the operation.Discussion We discuss the clinical features and neuroradiological findings of this rare condition.     

ADEM: literature review and case report of acute psychosis presentation

Acute disseminated encephalomyelitis is a monophasic, immune-mediated disorder that produces multifocal demyelinating lesions within the central nervous system. It is characterized clinically by the acute onset of neurologic abnormalities, including varying degrees of mental state changes ranging from drowsiness to coma. It is unusual for the illness to present as an isolated acute psychosis. The case of a 14-year-old female with biopsy-confirmed acute disseminated encephalomyelitis, who was initially diagnosed with an acute psychiatric disorder, is presented, and published reports on this unusual manifestation are reviewed. A Medline database search was performed from 1965 to 1999, using the terms acute disseminated encephalomyelitis, postvaccinal encephalomyelitis, postinfectious encephalomyelitis, and measles encephalomyelitis, combined with the terms psychosis, psychiatric disorder, and behavioral disorder. Selected cross-referenced reports were also reviewed. Nine patients were identified who presented with acute psychosis. We conclude that, although rare, acute disseminated encephalomyelitis can present as an acute psychosis. This immune-mediated condition should be included in the differential diagnosis of neurologic disorders presenting as a psychiatric illness.     

左侧三叉神经痛、面肌痉挛、舌咽神经痛及原发性高血压病1例报告并文献复习

目的探讨原发性三叉神经痛、面肌痉挛、舌咽神经痛及原发性高血压病发病的机制。方法报告1例行显微外科手术治疗的男性病人，并结合文献进行分析。结果该病例与相应脑神经根部动脉性压迫和蛛网膜增厚、黏连之间存在因果关系。行显微血管减压术及神经根选择性切断术后即刻及随访期间，病人三叉神经痛、面肌痉挛、舌咽神经痛均消失，血压恢复正常，无特殊并发症发生。结论同侧原发性三叉神经痛、面肌痉挛、舌咽神经痛及原发性高血压病极为罕见，其与脑神经根部血管压迫和蛛网膜增厚、黏连之间具有一定的因果关系；显微血管减压术并神经根选择性切断术是有效的治疗方法。     

Juvenile amyotrophic lateral sclerosis with unusual presentation: a case report

A case of juvenile amyotrophic lateral sclerosis with wasting confined to the distal part of one lower limb and the proximal part of the contralateral upper limb is being presented. A brief review of the literature is carried out.     

成人苯丙酮尿症一例

目的探讨成人苯丙酮尿症(PKU)的病因、临床表现、影像学特点。方法分析1例成人PKU的临床表现、影像学表现、检查及治疗结果。结果本例成人PKU患者主要表现为行走不稳、智力减退,影像学表现主要为双侧脑室旁脱髓鞘。查血液苯丙氨酸浓度升高,尿液蝶呤检测提示6-丙酮酰四氢生物蝶呤合成酶缺乏。按PKU给予低苯丙氨酸饮食,对症支持治疗。随诊7年时,患者血液苯丙氨酸水平正常,但患者临床症状及头颅影像学与入院时比较无明显变化。结论 PKU起病隐匿,临床表现多样,常引起不可逆的中枢神经系统脱髓鞘改变。新生儿疾病筛查虽然是重要诊断手段,但还需对PKU进行鉴别诊断及分型,并根据分型采取不同治疗方法,改善患者预后。     

Neuroblastoma in an adult causing spinal cord compression: report of a case and review of the literature

Adult neuroblastoma (ANB) is an uncommon malignancy, there being only 42 reported cases in the world literature. The purpose of this report is to present a case of ANB with spinal cord compression by an intraspinal component, and review methods of diagnosis and treatment. NB is one of the most frequent childhood malignancies (90% of the patients are less than 10 years old) and both pathologists and clinicians tend to exclude it from differential diagnosis when the patient is an adult. When the tumour is located in the paraspinal region, local extension through the intervertebral foramen into the spinal canal can occur in a dumbbell fashion, a pattern of growth first described by Weber in 1856.     

Limb-shaking transient ischemic attacks in an adult PHACE syndrome: a case report and review of the literature

PHACE syndrome is a neuro-cutaneous syndrome characterized by malformations of the Posterior fossa, facial Hemangiomas, Arterial anomalies, Cardiac anomalies, and abnormalities of the Eye. The arterial abnormalities usually involve the cervical and cerebral vasculature and include congenital abnormalities and progressive cerebral vasculopathy. The progressive cerebral vasculopathy leads to increased risk for arterial ischemic stroke (AIS) in patients with PHACE syndrome. Here we described the clinical neurological sequelae, the malformation of brain, the cervical and cerebral vasculopathy in a 23-year-old female of PHACE syndrome. Besides, she presented AIS with limb-shaking transient ischemic attack, a rare clinical presentation of AIS in patients of PHACE syndrome.     

Clinical analysis for an unusual large cystic meningioma: case report and review of the literature

The authors report the case of a 17-year-old boy with an unusual large cystic meningioma (Nauta type II) in the right hemisphere. The imaging appearances of this patient were very unusual. The shape of the huge cyst was crescentic and similar to subdural hematoma. It lay between the dura and the solid tumor parts. In addition there was a small intracystic nodule attached to the cyst wall. The patient underwent a right hemisphere craniotomy. At surgery it was found that the cyst contained a large amount of xanthochromic fluid and some semitransparent serumlike sediment. The intracystic nodule was proved to be necrotic substance without tissue and cell structure. Histological examination displayed an anaplastic meningioma, of which the cyst wall also consisted of meningioma tissue. To the best of the authors' knowledge, such an unusual case of cystic meningioma has not been reported. The authors review the literature with reference to intratumoral cyst associated with meningiomas, analyze the unusual imaging appearances of this patient, and explore the mechanism of cyst formation. The mechanism of cyst formation associated with meningiomas is not perfectly understood. Intratumoral cyst formation may be attributed to microcystic degeneration, ischemic necrosis, intratumoral hemorrhage, transudation and secretory changes within the tumor.     

Lateral medullary infarction with cardiovascular autonomic dysfunction: an unusual presentation with review of the literature

Purpose

We report an unusual case of lateral medullary infarction presenting with orthostatic hypotension with pre-syncope without vertigo or Horner’s syndrome.

Methods

Case report with review of the literature.

Results

A 67-year-old man presented with pre-syncope and ataxia without vertigo. Initial brain CT and MRI were normal. Neurological evaluation revealed right-beating nystagmus with left gaze, vertical binocular diplopia, right upper-extremity dysmetria, truncal ataxia with right axial lateropulsion, and right-facial and lower extremity hypoesthesia. Bedside blood pressure measurements disclosed orthostatic hypotension. He had normal sinus rhythm on telemetry and normal ejection fraction on echocardiogram. A repeat brain MRI disclosed an acute right dorsolateral medullary infarct. Autonomic testing showed reduced heart rate variability during paced deep breathing, attenuated late phase II and phase IV overshoot on Valsalva maneuver, and a fall of 25 mmHg of blood pressure at the end of a 10-min head-up tilt with no significant change in heart rate. These results were consistent with impaired sympathetic and parasympathetic cardiovascular reflexes. He was discharged to acute rehabilitation a week later with residual right dysmetria and ataxia.

Conclusion

Lateral medullary infarctions are usually reported as partial presentations of classical lateral medullary syndrome with accompanying unusual symptoms ranging from trigeminal neuralgias to hiccups. Pre-syncope from orthostatic hypotension is a rare presentation. In the first 3–4 days, absence of early DWI MRI findings is possible in small, dorsolateral medullary infarcts with sensory disturbances. Physicians should be aware of this presentation, as early diagnosis and optimal therapy are associated with good prognosis.

     

Suprasellar cystic meningioma: unusual presentation and review of the literature

We report on a case of suprasellar cystic meningioma. The magnetic resonance imaging of this tumor resembled that of a craniopharyngioma. The definitive diagnosis of meningioma was made only after histopathological confirmation. We conclude that preoperative studies are often equivocal. The possibility of meningioma should be considered in the diagnosis of any intracranial neoplasms with radiological and surgical evidence of a cystic lesion.     

Obstipation as a paraneoplastic presentation of small cell lung cancer: case report and literature review

Paraneoplastic symptoms caused by abnormal gastrointestinal motility may be the initial manifestation of small cell lung cancer (SCLC). We report a case of a 63-year-old woman who presented with progressive constipation culminating in obstipation, and associated symptoms of more widespread dysmotility. A paraneoplastic syndrome was suspected. The only abnormality on chest computed tomography was a minimally enlarged paratracheal lymph node. Positron emission tomography demonstrated increased activity in the lymph node. The antinuclear neuronal antibody titer was elevated. Bronchoscopy with transtracheal biopsy confirmed the diagnosis of SCLC. One year after diagnosis, the patient had progressive symptoms of intestinal obstruction, and ultimately feculent vomiting. On abdominal radiography, colonic sitz markers ingested a year earlier were in virtually the same positions as after ingestion. Palliative colectomy with ileostomy was performed. The myenteric plexus in the terminal ileum and colon showed infiltration by a mixture of B-cell and T-cell lymphocytes and plasma cells, and no gross neuronal abnormalities. We review the clinical and pathologic features, clinical course, and management of paraneoplastic pseudoobstruction.     

An uncommon initial presentation of snake bite-subarachnoid hemorrhage: A case report with literature review

Snake bites are very common in India, particularly in West Bengal. Snake bite can cause various hematological, neuromyopathical complications. It can be very fatal if not detected and treated early. Timely intervention can save the patient. We are reporting a case of hematotoxic Russell viper snake bite presented with subarachnoid hemorrhage. Patient was successfully treated with antivenom serum (AVS) along with other conservative management. Subarachnoid hemorrhage as an initial presentation in viper bite is very rare and we discuss the case with proper literature review.     

Seizures and EEG findings in an adult patient with DiGeorge syndrome: A case report and review of the literature

This is the first case report to describe the EEG findings in a patient with DiGeorge syndrome who survived into adulthood. The patient developed generalized tonic–clonic seizures when she was 9 years old and these were associated with hypocalcemia. Despite treatment with calcium, seizures persisted and the patient required antiepileptic medications. She was eventually controlled with oxcarbazepine. An MRI of the head was normal. An EEG showed independent spike and wave discharges emanating from the left temporal and right frontal region. The presence of focal findings on EEG, the lack of complete response to calcium therapy, and the need for antiepileptic drug therapy indicate that some of these patients may be inherently predisposed to developing epilepsy.