Multiple intracranial enterogenous cysts.

Abstract

SUMMARY The case of a 40-year-old woman with increasing ataxia is described. Although the clinical presentation and evoked response studies raised the possibility of multiple sclerosis, further investigation revealed multiple cystic intracranial lesions. Surgical excision of one of the lesions relieved the patient's symptoms. Histological examination revealed that this was an enterogenous cyst. Although single cysts of this type have rarely been reported occurring in the posterior cranial fossa, the occurrence of multiple lesions, some in the supratentorial compartment, appears to be unique. Case reportA 40-year-old-lady presented with a four month history of progressive difficulty in walking. She had become progressively more unsteady on her feet and had also noticed increasing stiffness in her legs with a tendency to spontaneous jerking of her legs when resting. For two months prior to presentation she had been aware of weakness in her arms when carrying heavy objects. She had had some hesitancy of micturition but no urgency or incontinence. There were no ocular or sensory symptoms and apart from occasional episodes of migraine, there were no headaches suggesting raised intracranial pressure and no systemic symptoms. Prior to her presenting illness, in childhood, a small "cyst" had been excised from her scalp in the occipital region. Regrettably no further information was available regarding the nature of this lesion. There was a history of Huntington's disease in her maternal family but her mother had died aged 62 years without manifesting this condition. Our patient had two older and two younger siblings and three children, all of whom were perfectly well. Examination revealed no systemic abnormalities. Examination of the scalp revealed no evidence of a cutaneous sinus. There were no involuntary movements and higher cerebral function was intact. The visual fields, fundi and acuity were normal. Sustained first degree horizontal nystagmus was present on lateral gaze to both sides, the maximum amplitude was on gaze to the right. The rest of the cranial nerves were intact and Radiographs of the chest, skull, dorsal and lumbar spine were normal. Radiographs of the cervical spine revealed failure of fusion of the posterior arch of the atlas but no other abnormality. Pattern reversal visual evoked responses (VERs) were abnormal with symmetrical delay and dispersion of the major components. Somatosensory evoked responses from median nerve stimulation at the wrist were normal. Brainstem auditory evoked responses (BAERs), however, were abnormal, being significantly asymmetrical (right V-I 377ms, left V-I 4-6ms), this delay occurring between waves III and IV. An electroencephalogram (EEG) revealed a significant generalised excess of theta and delta activity, which was sharply contoured in the left temporal region and of highest amplitude in both frontal regions. Cranial computed tomographic (CT) scans (fig 1) revealed a large, low attenuation lesion (+ 2 to -139 Hounsefield units) in the region of the fourth ventricle ...

Intraparenchymal Supratentorial Neurenteric Cyst

“…2,8,[20][21][22][23] Ten cases of supratentorial neurenteric cysts have been previously reported. Cyst locations include the parasellar/suprasellar region, 14,17 the septum pellucidum, 16 the third ventricle, 3 the orbital apex/superior orbital fissure, 15 the optic nerve, 18 and the extraaxial anterior cranial fossa 12,13,19 (Table). None of these cysts was contained within the brain parenchyma.…”

“…1 0 , 11 S u p r a t e n t o r i a l neurenteric cysts are very rare and none of the cysts in those cases reported to date have been located within the brain parenchyma. 3,[12][13][14][15][16][17][18][19] We report a case of a 35-year-old female who underwent successful excision of an intraaxial right frontal lobe neurenteric cyst. To our knowledge, this is the first case report of A B S T R A C T: B a c k g r o u n d : Neurenteric cysts are congenital cysts of the central nervous system that are believed to be of endodermal origin.…”

Intracranial neuroenteric cysts: A concise review including an illustrative patient

“…This is in contrast to previously published series which have suggested either male or female predominance. 77,95,96,107 The age at presentation of intracranial NC is later than that of intraspinal cysts, possibly due to the lower tolerance of local mass effect on the spinal cord. A total of 61 (44%) NC in reported patients were located within the posterior fossa with 47 (34%) extending from the posterior fossa to the cervicomedullary junction.…”

Fine-needle aspiration diagnosis of enterogenous cyst of the orbit: A case report

“…9 This case is the only one in the orbital apex. 2 Enterogenous cysts, including those arising intracranially, are difficult to differentiate from other cystic lesions.…”

“…7 Cysts lined by a simple cuboidal or columnar epithelium are labeled group A, and cysts with additional features such as mucous glands and smooth muscle, or an ependymal lining, are labeled group B and C, respectively. Most intracranial enterogenous cysts would be classified as group A. Intracranial enterogenous cysts are extremely rare, 2,5,6,[8][9][10] most being located in the posterior fossa. 2,9,11 A single case has …”