Nitaya Suwanwela

Combined neuroepithelial (colloid) cyst and xanthogranuloma (xanthoma) in the third ventricle

A case is reported of a combined neuroepithelial cyst and xanthogranuloma of the choroid plexus in the third ventricle of a 22-year-old woman. It is suggested that proliferated neuroepithelial cells lining the cyst enter the fibrous wall through the disrupted basal lamina, and then become xanthomatous cells. Disintegration of these foamy epithelial cells releases lipids and other materials into the cyst wall, provoking a response of macrophages and multinucleated giant cells of foreign-body type. A xanthogranuloma is then formed. The origin of “colloid” cysts is from neuroepithelium; these cysts arise from both ependyma and choroid plexus. Those cysts arising in or near the floor of the third ventricle may originate in stomodeal epithelium, but a distinction cannot be made from neuroepithelial cysts by presently available methods.

Takayasu arteritis in Thailand: clinical and imaging features

PURPOSE To present and evaluate the clinical and imaging features of patients with Takyasu arteritis in Thailand. MATERIALS AND METHODS Clinical and angiographic findings were studied in 63 patients with Takayasu arteritis collected at Chulalongkorn Hospital Medical School, Bangkok. Imaging features including CT scan in 15 patients, magnetic resonance imaging in 14 patients and magnetic resonance angiography in ten patients were evaluated and compared to angiography. RESULTS Among 63 patients there were 43 females and 20 males with the ratio female to male 2.15:1. The most common age groups were in the third and fourth decades of life. The most common clinical findings were hypertension and absence or weakness of pulses. Elevated erythrocyte sedimentation rate was found in 50%. For angiography, the most common vessel affected was the abdominal aorta (20%). The second and third most common sites were renal arteries (18.7%) and the subclavian arteries (14.3%). Coronary artery involvement was found in two out of eight patients. No pulmonary involvement was found in eight cases studied by pulmonary angiography. For classification of angiogram, the most common type of involvement (66.7%) was extensive involvement of the aorta and branches (type V of new classification established from International Conferences on Takayasu arteritis in Tokyo 1994). No patient was found to have only involvement of brachiocephalic branches or ascending aorta and brachiocephalic branches. CT scan showed calcification in the vessel walls (8/15 cases) which helped in diagnosis of the disease. Wall thickness, surrounding tissue, appearance of the vessel and intraluminal thrombus could be well seen. Magnetic resonance imaging added more detail in multiple planes and could demonstrate vascular flow. High detection rate of the lesions (94.8%) was found for the aorta. The study was less sensitive for smaller vessels. Magnetic resonance angiography showed better correlation of the findings of brachiocephalic arteries compared to angiography. Subclavian steal syndrome could be diagnosed. For renal arteries, MR angiography could correctly demonstrate occlusion of renal artery in six out of eight cases. In the other two cases only stenoses of the vessels were found on angiograms. One renal arterial stenosis on MR angiography was confirmed by angiography. CONCLUSION Takayasu arteritis could be found in Thailand. Clinical features were almost the same as in reports from other countries. Various forms of arterial involvement were demonstrated by imaging modalities. Angiography remained the best method for detection and diagnosis of Takayasu arteritis in correlation with clinical and laboratory findings. Although CT scan, magnetic resonance imaging and magnetic resonance angiography were limited and less sensitive for detection of the lesions, they were useful as noninvasive modalities showing more details of intraluminal clot, vascular wall and surrounding tissue which could be correlated with pathological findings and pathogenesis of the disease. Our study suggests that MR imaging and MR angiography can be screening and diagnostic techniques for patients with suspected Takayasu arteritis.

Takayasu arteritis in Thailand

SummaryTakayasu arteritis is a disease of worldwide distribution. Geographic difference in sex incidence, anatomical distribution, and the type of lesion is observed. Hypertension is very common in the present series, as well as in combination with absent or deficit of peripheral pulses. These symptoms correlate well with arteriographic findings of brachiocephalic and renal artery obstructive lesion. While aneurysm and stenotic lesions have a predilection in the abdominal aorta, stenotic lesion of the thoracic aorta occurs more commonly than aneurysm. However, aneurysm of the aorta as well as of the brachiocephalic arteries is seen more frequently than in the reports, of others.The presence of “funnel-shape” resulting from renal artery obstructive lesion and dilatation or aneurysm of contiguous aorta was characteristic of Takayasu arteritis in our series.The material presented in this report reflects not only geographic variation but also the severe form of this disease. Total aortography, coronary arteriography and pulmonary arteriography, are of value not only for the diagnosis of Takayasu arteritis but also for demonstration of anatomical distribution, severity and type of lesion.

Primary intraorbital extraocular primitive neuroectodermal (neuroepithelial) tumour.

A case is reported of primary primitive neuroectodermal (neuroepithelial) tumour occurring in the right orbit of a 52-year-old man. The intraorbital extraocular location is unique for this kind of neoplasm. The malignant tumour was differentiated into primitive neuroepithelial, ependymal, and oligodendroglial cells. The neuroglia was identified by localisation of cytoplasmic glial fibrillary acidic protein. It is suggested that this primary intraorbital, extraocular, primitive neuroectodermal tumour with neuroglial differentiation is akin to the primitive neuroectodermal tumours of the neuraxis, including the cerebellar medulloblastomas, and to some peripheral nerve tumours known as malignant neuroepitheliomas, malignant ependymomas, and neuroblastomas. The ectomesenchymal remnant of the neural crest or ectopic neuroepithelium or both may have been the origin of the present tumour. Differentiation of the neuroectodermal component of the neural crest or heterotopic neuroepithelium or both would create a primitive neuroectodermal tumour with diverse neuroglial elements.

Posterior leukoencephalopathy following cisplatin, bleomycin and vinblastine therapy for germ cell tumor of the ovary

A 31‐year‐old female developed multiple episodes of grand mal seizures after combination chemotherapy with cisplatin, vinblastine and bleomycin for germ cell ovarian cancer stage Ic. The clinicoradiologic fea‐tures in this patient were consistent with posterior leukoencephalopathy, which is a rare complication of chemotherapy. Seizures were controlled by the anticonvulsive agent Dilantin (Pfizer, Khet Klongtoey, Bangkok) and she returned home without any permanent neurologic deficits. Follow‐up magnetic resonance imaging 2 weeks later showed complete resolution of the abnormalities. This syndrome has been previously reported following cisplatin‐based chemotherapy. Physicians should remain alert to the potential hazards of chemotherapy to the central nervous system. Risks and benefits should be seriously considered before starting treatment.

Takayasu Arteritis : ultrasonographic evaluation of the cervico-cerebral arteries

PURPOSE To present the ultrasonographic findings of the cervico-cerebral arteries in patients with Takayasu arteritis. MATERIALS AND METHODS We used duplex ultrasound to study the arteries in the neck including the common carotid artery (CCA), internal carotid artery (ICA), external carotid artery (ECA) and the vertebral arteries. For the intracranial arteries, transcranial Doppler ultrasound was used to study the blood flow velocities and directions in the middle cerebral artery (MCA), anterior cerebral artery (ACA), posterior cerebral artery (PCA), ophthalmic artery, basilar and vertebral arteries. The ultrasonographic findings were compared with magnetic resonance angiography (MRA) and standard angiography. RESULTS Four patients with the diagnosis of Takayasu arteritis were studied. One patient presented with ischemic optic neuropathy and was found to have absent radial pulse. Two patients presented with severe hypertension in young age. The other patient had accidental findings of unmeasurable blood pressure in both arms. One patient had abnormal carotid duplex ultrasound. He was found to have an occlusion of the right ICA. Thickening of the wall of the left ICA was also noted. Reduction of flow velocity in the MCA was demonstrated on TCD. There was evidence of collateral circulation from the contralateral carotid artery demonstrated by reversal of flow in the ipsilateral A1 segment of the ACA. MRA and angiography confirmed the ultrasound findings. Three patients had normal carotid duplex ultrasound but one of them was found to have a severe stenosis at the origin of the common carotid artery by MRA and angiogram. Absence of one vertebral artery was noted in three patients. MRA and angiogram confirmed the occlusion of the same vertebral arteries at their origins. In one patient, reversal of flow in the intracranial part (V4 segment) of the affected vertebral artery indicating collateral flow from contralateral vertebral and basilar arteries was demonstrated. CONCLUSION Ultrasonography could be used as an additional noninvasive procedure apart from CT angiography, MRI and MRA in patients with Takayasu arteritis. Occlusion and wall thickening of the neck artery could be detected by color coded B-mode and Doppler ultrasound. Transcranial Doppler was useful in evaluation of flow velocity and flow direction of the arteries around the circle of Willis.

Magnetic resonance images of brain stem infarct in periarteritis nodosa

A 35-year-old woman displayed unusual symptoms of progressive bulbar dysfunction. At necropsy, systemic necrotizing vasculitis and small pontine infarcts were demonstrated. The angiographic and magnetic resonance imaging findings are described. The latter investigation is a sensitive method for detecting such pontine lesions. To our knowledge, this case is the first report of magnetic resonance imaging findings of brain stem infarct in a patient with periarteritis nodosa.

Unilateral proptosis as a clinical presentation in primary angiosarcoma of skull.

A case is reported of unilateral proptosis of the left eyeball as a unique clinical presentation of primary angiosarcoma of the skull involving the sphenoid and frontal bones and roof of the orbit on the left side of a 32-year-old man. The intraosseous, extradural non-encapsulated, and friable tumour contained cytoplasmic factor VIII-related antigen. After complete removal of the tumour the patient has been well for 10 months of the follow-up period. This case brings the total number of reported primary angiosarcomas of the cranium to nine. All patients were male and most of them were young. The average age was 24 years. The frontal bone seems to be the site of predilection for cranial angiosarcomas. Surgical extirpation, radiation, or combined surgical removal and radiation have been used for treating angiosarcoma of the skull with variable results. It is suggested that angiosarcoma of the skull has a worse prognosis than angiosarcoma of bones other than the cranium because the patient may die of secondary neoplastic involvement of the brain.

Primary meningioma intimately related to skull: Case report and review of the literature

A report is made of a primary meningioma intimately associated with the left sphenoid ridge and temporal bone of a 41-year-old woman. The lesion produced hyperostosis of the involved bones, ipsilateral proptosis, and reduced vision for 4.5 years. The radiotherapy yielded little improvement of the exophthalmos and vision. Review of 125 reported meningiomas intimately related to the skull and presenting primarily with hyperostosis and minimal soft tissue component, including the current case, disclosed that all but one affected the cranium proper, particularly the middle ear, temporal bone, frontal bone, and parietal bone. The exceptional one was in the mandible. They tended to occur between the fifth and seventh decades of life with the peak occurrence between the fifth and sixth decades. The average age was 46 years for overall. The female-to-male ratio was 2:1. Six of 125 primary meningiomas closely associated with the skull were malignant and two of these spread to the lungs. The pulmonary metastasis was spontaneous in one case.

Postnatal growth failure, microcephaly, mental retardation, cataracts, large joint contractures, osteoporosis, cortical dysplasia, and cerebellar atrophy

We describe two sibs with postnatal‐onset growth deficiency, microcephaly, cataract, prominent supraorbital ridge, large joint contractures, severe osteoporosis, cortical dysplasia, cerebellar atrophy, and mental retardation. The combination appears to constitute a previously undescribed syndrome inherited in an autosomal recessive pattern.