Epidermoid cysts of the pineal region

Localization of epidermoid cysts to the pineal region is rare. The 7-year-old boy now reported presented with an 18-month history of progressive ataxia. CT and MRI scans demonstrated a 2.5×2.5 cm cyst at the pineal region with obstructive hydrocephalus. At surgery via an occipital transtentorial approach, a characteristic “pearly tumour” was encountered, and complete resection was achieved. We present the management of this child with pineal region epidermoid cyst and review 11 cases reported in the literature since 1968. In all, 8 of the 12 patients were males. The age at the time of diagnosis ranged from 7 years to 69 years. Parinaud's syndrome and hydrocephalus are the most common presenting findings. All but 1 patient underwent direct surgical resection; 1 had stereotactic decompression. Surgical treatment brought about complete resolution of the presenting symptoms and signs in 10 of the 12 cases. One patient had persistent upgaze palsy. One patient died from progression of the pineal region mass. This patient presented with hemiparesis, which is a marker of clinical aggressiveness. The authors advocate direct surgical attack as opposed to stereotactic diagnosis and aspiration to: (1) obtain maximal resection and thereby limit the potential for recurrence and delayed complications of the cyst; (2) possibly avoid shunt placement in patients who present with hydrocephalus; and (3) decrease the likelihood of sampling error. Received: 19 June 1998     

Ictal prosopagnosia and epileptogenic damage of the dominant hemisphere. A case history

This case report concerns a patient whose episodes of prosopagnosia appeared to be epileptic manifestations arising from the left occipital lobe. The underlying pathology, as revealed by radiologic and surgical findings, was an atrophic-hypoplastic lesion of the left hemisphere, more prominent in the posterior sections, likely dating from early life. Removal of an arachnoidal cyst of the occipital pole and excision of the damaged gray matter were followed by the disappearance of the prosopagnosic seizures for the two years between the date of the surgical intervention and the publication of this article.     

Acute hydrocephalus in a child with a third ventricle arachnoid cyst and coincidental enteroviral meningitis

We present a 2.5-year-old child suffering from acute hydrocephalus. First, the child was diagnosed with aseptic viral meningitis. The PCR of the cerebrospinal fluid (CSF) was positive for enterovirus. Subsequently, MRI revealed that the hydrocephalus was caused by a cyst in the third ventricle. During ventriculoscopy, the cyst had all aspects of an arachnoid cyst. An endoscopic fenestration and partial removal of the cyst was performed, combined with a ventriculocisternostomy. The coincidental finding of viral meningitis and a third ventricle arachnoid cyst in a patient with acute hydrocephalus has, to our knowledge, not been described in literature before. If there is a relation between the enteroviral meningitis, the arachnoid cyst (possibly causing a pre-existing subclinical hydrocephalus) and the rapidly evolving neurological deterioration, remains speculative. Proposed mechanisms, by which the viral meningitis could accelerate the disease process, are slight brain swelling or increased CSF production. This rare combination of diagnoses could also be coincidental.     

Posttraumatic Giant Extradural Intradiploic Epidermoid Cysts of Posterior Cranial Fossa : Case Report and Review of the Literature

We reported a unique case of posttraumatic giant infratentorial extradural intradiploic epidermoid cyst. A 54-year-old male, with a previous history of an open scalp injury and underlying linear skull fracture in the left occipital region in childhood, presented with a painful subcutaneous swelling, which had been developed gradually in the same region and moderate headache, nausea, vomiting and cerebellar ataxia. The duration of symptoms on admission was 3 months. Imaging studies revealed occipital bone destruction and giant extradural intradiploic lesion. The preoperative diagnosis was giant infratentorial extradural intradiploic epidermoid cyst. Surgery achieved total removal of the lesion, which was histologically confirmed and the postoperative course was uneventful. To our knowledge, this is the first case of giant infratentorial extradural intradiploic epidermoid cyst with a traumatic etiology described in the literature.     

Choroid plexus cyst of the left lateral ventricle with intermittent blockage of the foramen of Monro, and initial invagination into the III ventricle in a child

A cyst of the choroid plexus of the left lateral ventricle with intermittent blockage of the foramen of Monro and initially with invagination of the III ventricle in a child is described. In a 6-week-old boy a ventriculoatrial shunt was implanted for correction of an active asymmetrical hydrocephalus of unknown origin. When he was 3 months of age a water-soluble contrast CT ventriculography revealed a noncolloid cyst localised predominantly in the upper portion of the III ventricle. At that time the ventricular catheter obstructed with choroid plexus was removed; new bilateral catheters in a parieto-occipital region were implanted. In the course of the next 4 years, first the atrial catheter had to be extracted and then the peritoneal catheter was changed, in both cases because of obstruction. Periods of normal life alternated with periods of transient and intermittent symptoms of increased intracranial pressure, papilloedema, and myoclonic jerks. Repeated computed tomography (CT) and magnetic resonance imaging (MRI) showed stabilised hydrocephalus with an enlarged left lateral ventricle. When the boy was 16 years old MRI revealed a choroid plexus cyst in the left lateral ventricle 2 cm in diameter, with a ball-valve type of obstruction of the foramen of Monro. CT stereoendoscopic resection of the wall of a large cyst filled with cerebrospinal fluid was performed, and two additional adnexal small cysts were coagulated using the bipolar coagulator, Diomed 25 laser and scissors; the symptoms then regressed, except for superior bilateral altitudinal anopsia. Light and electron microscopy of the cyst wall is reported. The cyst was composed of collagenic connective tissue lined with a basal lamina lacking in epithelial cells. The preoperative and postoperative MRI are presented. Choroid plexus cysts localised in the anterior part of lateral ventricles are very rare, and all reported cases have been in male patients. According to the literature our case is only the third ever described in a child. Received: 9 March 1998 Revised: 12 May 1998     

Endoscopic management of posterior fossa arachnoid cyst in an adult: case report and technical note

The authors report a case of large arachnoid cysts of the posterior fossa treated by endoscopic surgery. A 40-year-old man was admitted with a 3-month history of headache and progressive gait imbalance, with no significant medical history. At the time of admission, neurological examination revealed no abnormalities except for tandem imbalance. Brain computerized tomography (CT) scan and magnetic resonance imaging (MRI) revealed a posterior fossa arachnoid cyst (PFAC) causing brain stem compression accompanied by hydrocephalus. The patient was treated by endoscopic fenestration of the cyst with a paramedian suboccipital transcortical approach. Postoperatively the patient's complaints showed improvement and he was discharged with no complications. Follow up MRI showed a decrease in the cyst's size and the hydrocephalus with decompression of the brain stem.     

Post-traumatic intradiploic leptomeningeal cyst of the posterior fossa in an adult

Intradiploic cysts in the posterior fossa are rare. We report a post-traumatic intradiploic leptomeningeal cyst in an adult and review the diagnosis and pathogenesis of this lesion.A 28-year-old woman presented with a headache and a hard mass in the occipital region. She had a history of head injury as she had fallen from a height at the age of 18 months. CT scans and MRI revealed an expanding intradiploic cyst with the density of cerebrospinal fluid (CSF) and thinning of the inner and outer tables. A bony defect of the inner table connecting to the cisterna magna was also visualized. At surgery, we detected free communication of the CSF between the cystic cavity and the subarachnoid space of the posterior fossa via a defect in the dura and inner table. The dural defect was sutured primarily and covered with the autogenous outer table.An intradiploic cyst of the occipital bone can be detected in adults and might be caused by remote head injuries during childhood. Prompt surgical repair of the dural and bony defect in an adult patient has an excellent prognosis.     

Supratentorial cystic hemangioblastoma with infratentorial extension—a unique location and a rare infant case

Introduction Supratentorial occurrence of hemangioblastoma is an exceedingly rare event. Even rarer is the occurrence of a supratentorial hemangioblastoma in infancy.Case report We hereby report the case of an 18-month-old girl who presented with irritability, increasing head size, and an open fontanelle. MR scans demonstrated triventricular hydrocephalus and a cystic mass within the left lateral ventricle. There was an 18×15×13 mm enhancing nodule along the medial aspect of the cyst. The cystic mass was mainly supratentorial but there was a caudal extension through the tentorial incisura that compressed the cerebellum. At surgery, the content of the cyst was xanthochromic. Enhancing medial nodule was extremely vascular and was extirpated totally. The pathological diagnosis was reticular variant of hemangioblastoma. Conclusion The child is well at 4 years of age. An extensive review of the English literature revealed only three such cases. All three cases survived the operation.     

Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum)

OBJECTIVE: Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is a rare hamartomatous lesion of the cerebellar cortex. The pathogenesis of the disease is still poorly understood. Lhermitte-Duclos disease was recently considered to be part of a multiple hamartoma-neoplasia syndrome (Cowden disease). We add two further cases to this rare entity. PATIENTS: A 24-year old woman presented with occipital headaches, blurred vision, diplopia and ataxia of gait. Physical examination revealed turricephaly. The second patient was a 37-year old woman, who presented with progressive occipital headache with nausea and vomiting. Physical examination revealed congenital facial asymmetry. Computed tomography and NMR-imaging, respectively demonstrated a space occupying mass of a cerebellar hemisphere in both cases. RESULTS: Suboccipital craniotomy and complete removal of the infratentorial tumour were performed in both patients. Histopathological findings clinched the diagnosis of Lhermitte-Duclos disease. Postoperative course was uneventful in the first and complicated by progressive occlusive hydrocephalus in the second patient, necessitating permanent surgical shunt drainage. Both patients were discharged free of complaints. CONCLUSIONS: Dysplastic cerebellar gangliocytoma is commonly associated with progressive mass effects in the posterior fossa and typically presents with headaches, cerebellar dysfunction, occlusive hydrocephalus and cranial nerve palsies. The disease usually manifests in young adults, but the age at presentation ranges from birth to the sixth decade. There is no sex predilection. NMR-imaging became a useful clue to the diagnosis within the last decade. Therapy consists of decompression of the posterior fossa by total surgical removal of the tumour mass.     

Neuropathological analysis of an asymptomatic adult case with Dandy-Walker variant

The Dandy-Walker (DW) complex is a rare posterior fossa malformation, usually observed during the prenatal period or the early infancy. Clinically, it is characterized by mental retardation, seizures, cerebellar ataxia as well as symptoms of hydrocephalus. Structural imaging reveal a hypoplasia or agenesis of the cerebellar vermis, enlargement of the fourth ventricle with a posterior fossa cyst. Additional neurodevelopmental changes such as agenesis of the corpus callosum, lissencephaly and cortical dysplasia are also present. We report the first neuropathological analysis of an adult asymptomatic DW case. Brain computerized tomography showed a massive posterior fossa cyst and hypoplasia of the cerebellum. An Ehlers-Danlos syndrome type IV characterized by repetitive intestinal perforations and a saccular aneurysm on the left posterior communicating artery was also present. Macroscopic brain examination revealed hypoplasia of both cerebellar hemispheres and posterior part of the vermis, as well as dilatation of the fourth ventricle without hydrocephalus. The posterior fossa cyst wall was formed by an external arachnoid layer, middle layer with loose connective tissue and an internal layer of ependymal cells. There were two foci of cerebellar cortical dysplasia but no ectopic neurons, neuronal loss or gliosis in both cerebellum and cerebral cortex. No vascular or significant neurodegenerative lesions were observed. In comparison with previous reports in DW infants, this adult case displayed milder brain abnormalities compatible with a diagnosis of DW variant. The preservation of the cortical cytoarchitecture as well as the paucity of additional neurodevelopmental changes may explain the absence of clinical expression.     

Neurozystizerkose Aktuelle Literaturübersicht anhand einer Langzeitbeobachtung zweier klinisch distinkter deutscher Erkankungsfälle

Neurocysticercosis, caused by Taenia solium larvae (cysticerci), is the most common parasitic infection of the human CNS Worldwide. In Germany its appearance is rare. Here we report two cases of neurocysticercosis which we followed over a period of 4-6 years. The first patient acquired neurocysticercosis in Germany. On admission he suffered from papilledema, partial seizures and a mild psychotic disorder. MR-tomography showed an internal hydrocephalus and multiple contrast enhancing parenchymal cysts. In the course of the disease a giant cyst within the left temporal pole developed and was exstirpated neurosurgically. The persistent internal hydrocephalus required ventriculoperitoneal shunting. Therapy with Praziquantel led to a clinical improvement, however, repeated analysis of the cerebrospinal fluid documented persistent inflammation. With the aid of a contrast enhanced three-dimensional (3D) ultrasound imaging technique we demonstrated increased cerebral perfusion surrounding one cyst. This may be interpreted as evidence for persistent disease activity. The second patient presented with repeated episodes of cysticercotic encephalitis, which is rarely described in the literature (incidence 1%). Clinical features, laboratory findings, diagnosis, and therapy of neurocysticercosis will be presented together with the pitfalls of the two described cases. Our cases will be compared to previous reports on clinical findings in neurocysticercosis.     

A huge occipital osteoblastoma accompanied with aneurysmal bone cyst in the posterior cranial fossa

Osteoblastoma is an infrequent benign tumor and skull involvement is extremely rare. The occipital bone is much less frequently involved. We report an unusual case of a huge occipital osteoblastoma with aneurysmal bone cyst in the posterior cranial fossa of a 20-year-old young man. MRI scan and CT three-dimensional reconstruction revealed that the tumor (approximately 8.5 cm x 6 cm x 5 cm) occupied nearly half volume of the posterior cranial fossa. The cerebellum, the forth ventricle and brain stem were compressed remarkably and hydrocephalus was noted. The tumor mass was multiloculated cystic and highly vascular. Excessive bleeding occurred during operation and total removal was achieved. The patient was symptom-free and MRI scan revealed no evidence of recrudescence after a follow-up period of 18 months. Pathological examination revealed that the lesion was a benign osteoblastoma with secondary aneurysmal bone cyst (ABC). ABC constitutes a highly vascular lesion while complete surgical resection is often impeded by excessive intraoperative bleeding. Preoperative embolization may reduce the risk. Despite the benign nature of osteoblastoma, local recurrence after incomplete resection is not rare and malignant transformation during recurrence may occur even after adequate surgery. Our case confirms the findings of previous investigators that if osteoblastoma is removed completely, no recurrence is expected even without adjuvant irradiation and chemotherapy.     

Usefulness of spinal drainage for post-traumatic external hydrocephalus: report of two cases

An 8-year-old girl presented with symptoms of increased intracranial pressure on the 18th day after removal of an occipital epidural haematoma. A 69-year-old woman exhibited the same symptoms on the 6th day after the removal of a left frontal subdural haematoma. Computed tomography (CT) revealed that both patients were suffering from external hydrocephalus. Spinal drainage, maintained for 18 days in the former case and for 7 days in the latter, not only relieved those symptoms but also cured the external hydrocephalus. Recurrence of external hydrocephalus has not been observed in either case for several years since discontinuation of spinal drainage. Usefulness of spinal drainage for post-traumatic external hydrocephalus is discussed.     

Diffuse axonal injury (DAI) in an autopsy case of head trauma with long survival]

The authors reported a clinico-pathological case survived 11 months after a traffic accident. A 41-year-old man had been hit by a motor car and was found in a state of semicoma. On admission, his consciousness level was III-100 to 200 (Japan Coma Scale). Pupils were isocoric; light reflex was present. Linear fracture of occipital bone was disclosed by Skull X-ray and subarachnoid hemorrhage was revealed on CT scan. This comatose state, lasting 24 hours, slowly improved and eventually he presented the so-called Korsakoff's syndrome until his death. He could not recognized his relatives, only uttered some meaningless words. He was unable to obey simple verbal orders. The patient was incontinent and right pyramidal sign was positive. On repeated CT scans, cerebral ventricles gradually increased in size; especially the enlargement of the fourth ventricle was remarkable. He expired of septic shock caused by bed sores. At autopsy brain weighed 1190 g. Old gloss contusional scars were observed on the bilateral frontal lobes including the orbital area and on the left temporal pole. Gliding contusions were revealed in the subcortical white matter beneath the left superior frontal convolution. Fibrillary gliosis was noted in this region, the deep white matter underlying the left temporal pole and the tissue surrounding the anterior horn of the left lateral ventricle. Nerve fibers were fragmented and lacerated at corpus callosum, anterior commissure and posterior limb of the left internal capsule. Bilateral pyramidal tracts showed mild myelin pallor at the brainstem. Loss of Purkinje cells were observed. This case would correspond to mile type of diffuse axonal injury proposed by Adams and Gennarelli. (ABSTRACT TRUNCATED AT 250 WORDS)     

First case report of neurobrucellosis associated with hydrocephalus

Brucellosis is a common zoonosis in many parts of the world, including Mediterranean and Middle Eastern countries. The disease is primarily related to occupations at risk, such as veterinarians, farmers, laboratory technicians, abattoir workers, and others working with animals and their products. Neurologic complications of brucellosis are quite rare, ranging from 1.7 to 10% of those infected. To date, no cases of neurobrucellosis with hydrocephalus have been reported. A 38-year-old right-handed farmer complained of headaches, nausea, vomiting, gait disturbance, and sweating for 2 days. He also complained of bilateral hearing loss of 4 months duration. On neurologic examination, dysmmetry, dysdiadochokinesis, ataxia on the left, and bilateral sensorineural hearing loss existed. On cranial MRI, a communicating hydrocephalus was noted. Because the patient consumed fresh sheep cheese and was a farmer, brucellosis was considered in the differential diagnosis. Brucella agglutination was positive with a 1/320 titer in the blood and a 1/80 titer in the cerebrospinal fluid. Ceftriaxone, doxycycline, and rifampicin were administered and by the fourth week of treatment, the ataxia was markedly improved, and the patient was able to walk without support. His cranial MRI demonstrated a total regression of the hydrocephalus. As a result, we suggest that neurobrucellosis should be considered in patients with hydrocephalus, especially if they live in an endemic area for brucellosis, even in the absence of other systemic signs.     

Alexia without agraphia following biopsy of a left thalamic tumor

Alexia without agraphia is a rare disconnection syndrome characterized by the loss of reading ability with retention of writing and verbal comprehension. We report a patient who developed alexia without agraphia after undergoing a biopsy for a malignant glioma involving the left thalamus. A 15-year-old right-handed male presented with 3 days of severe headache, and vomiting, and 1 month of blurry vision in his right visual field. Magnetic resonance imaging of the brain disclosed a large exophytic mass originating in the left thalamus, with mass effect and hydrocephalus. The patient underwent biopsy of the left thalamic mass via a transcallosal approach. Postoperatively, the patient complained of inability to read or identify letters. Examination revealed alexia without agraphia. The syndrome of alexia without agraphia can be rarely caused after surgery. A transcallosal procedure through the splenium of the corpus callosum may disrupt the visual association fibers traveling from the right occipital cortex to the left angular gyrus. In our case the syndrome occurred because of a preexisting right homonymous hemianopia resulting from a left thalamic tumor.     

Nocardia abscess during treatment of brain toxoplasmosis in a patient with aids, utility of proton MR spectroscopy and diffusion-weighted imaging in diagnosis

We report the case of a 30-year-old man with known HIV-positive status who developed, 4 months prior to admission, recurrent left partial motor seizures followed by left hemiparesis. At another hospital, contrasted CT scan of the head revealed right frontal hypodense lesion with mass effect and focal contrast enhancement. A small left occipital lesion was also present. HIV-associated brain toxoplasmosis was considered and phenytoin, pyrimethamine, clindamycin and antiretrovirals were administered. Hemiparesis improved but, 3 weeks prior to admission, he developed progressive headache and bilateral visual defects. Upon admission to our center, he was found with left homonymous hemianopsia, right hemiparesis and a large hypodense left occipital lesion on a head CT scan. Proton MR spectroscopy showed lactate at 1.3ppm, amino acids at 0.9ppm, and diffusion-weighted imaging (DWI) revealed hyperintensity at the lesion, suggesting a pyogenic abscess. Aspiration yielded purulent material and Nocardia asteroides grew in culture. The patient was treated with trimethoprim-sulfametoxazole and recovered with a mild visual field residual defect.     

A case of subtentorial ventricular diverticulum accompanied with choroid plexus papilloma in the lateral ventricle

Marked non-communicating hydrocephalus may rarely cause ventricular rupture producing either a dilated cystic cavity (ventricular diverticulum) or communication between ventricle system and subarachnoid space (spontaneous ventriculostomy). Ventricular diverticulum has been believed to be collection of cerebrospinal fluid which escaped beneath the pia mater after rupture of ependymal layers and cerebral parenchyma. We proposed herein to report a case of subtentorial ventricular diverticulum which accompanied with choroid plexus papilloma of the lateral ventricle. A nine year-old girl admitted to our hospital complaining of clumsiness of hands and walking, disability of reading, headache and vomiting. The neurological examination revealed alexia, papilledema, anisocoria, righ hemianopsia, weakness of right upper limb, and cerebellar ataxia. CT brain scan showed a large high density area at the trigone of the left lateral ventricle with non-communicating hydrocephalus and an extra-axial low density area in the posterior fossa. The medial space of the left trigone was especially ballooned and is just shifted above incisura tentoria by the tumor. The intraventricular tumor was totally removed by operation and proved to be benign choroid plexus papilloma microscopically. The subtentorial mass was confirmed to be a cyst contiguous to the medial trigone of the lateral ventricle, namely ventricular diverticulum. Three special features were recognized in this case. The first, this was the first example of ventricular rupture accompanied with tumor in the lateral ventricle reviewing all reports of both ventricular diverticulum and spontaneous ventriculostomy. The second, this ventricular diverticulum was not produced merely by hydrocephalus alone but mainly by direct effects of the tumor.(ABSTRACT TRUNCATED AT 250 WORDS)     

Primary nodular meningeal glioma mimicking metastatic tumor of the cerebellum with diffuse infra- and supratentorial leptomeningeal spread

A 62-year-old man presented with acute headache, blurred and double vision, nausea, and ataxia. Magnetic resonance imaging showed an enhancing mass on the inferior aspect of the right cerebellar hemisphere as well as a thin, widespread leptomeningeal enhancement and T2-weighted hyperintense lesions in the left occipital lobe and both thalami. Lumbar puncture revealed clusters of anaplastic cells. Therefore, metastatic tumor of unknown origin was suggested. Despite whole brain irradiation and intrathecal chemotherapy the patient deteriorated gradually and died four months later. Post-mortem examination of the brain revealed a nodular, high-grade astrocytic tumor within the subarachnoid space on the lower portion of the right cerebellar hemisphere. Diffuse leptomeningeal spread was noted, but cerebellar parenchyma was not infiltrated.     

Primary intracranial squamous cell carcinoma arising in an epidermoid cyst--a case report and review of literature

Primary intracranial squamous cell carcinoma is extremely rare, with most of the cases arising from malignant transformation of an epidermoid or a dermoid cyst. We report here a case of a 45-year-old male patient who presented with 1-month history of intermittent headache and recent onset of altered sensorium. Imaging revealed a midline posterior fossa mass lesion compressing the fourth ventricle and causing hydrocephalus. A provisional diagnosis of dermoid cyst was considered. Histopathological examination revealed a squamous cell carcinoma possibly arising from an underlying epidermoid cyst. This entity is being reported for its rarity.