1/9. Subacute diencephalic angioencephalopathy: biopsy diagnosis and radiological features of a rare entity.Subacute diencephalic angioencephalopathy (SDAE) is a rare and fatal disease of unknown etiology that involves the thalami bilaterally. To date, there have been four cases reported, in which the diagnosis was established only after post mortem examination of the brain. We report two male patients, ages 69 and 41 years, who presented with progressive dementia and somnolence. Radiological evaluation revealed enhancing lesions involving both thalami. The differential diagnosis included a number of neoplastic, inflammatory and vascular processes. In both cases, pathological evaluation of biopsy specimens suggested the diagnosis of SDAE. Despite supportive care, the disease progressed rapidly and both patients died within weeks after initial presentation. The diagnosis was confirmed at autopsy in both cases. SDAE is a rare cause of bithalamic disease that can be mistaken for a neoplasm as well as a number of conditions that necessitate different treatment choices. The histopathological findings can establish the diagnosis when combined with radiological and clinical information. This report emphasizes the utility of stereotactic biopsy in early diagnosis of SDAE.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/9. Transient crossed cerebellar diaschisis following thalamic hemorrhage.This report concerns a 65-year-old right-handed woman with cerebral hemorrhage who presented with mild right-sided hemiparesis. Computed tomography (CT) revealed hematoma in the left thalamus and compression of the posterior limb of the internal capsule by a brain edema surrounding the lesion. 99mTc-hexamethylpropyleneamine oxime (HMPAO) single photon emission computed tomography (SPECT) images obtained 4 days after onset showed hypoperfusion in the left thalamus containing a hematoma as well as contralateral cerebellar hypoperfusion to the supratentorial lesion, which is well recognized as crossed cerebellar diaschisis (CCD) after stroke. CT 14 days after the onset revealed reduction of the brain edema of the posterior limb of the internal capsule accompanied by gradual neurological improvement. SPECT obtained 14 and 28 days later showed that CCD had disappeared. In this case report, the authors discuss the disappearance of CCD due to transient edematous compression of the internal capsule following thalamic hemorrhage on serial 99mTc-HMPAO SPECT scans. CCD was possibly caused by the lesion confined to the posterior limb of the internal capsule, which anatomically constitutes the cerebropontocerebellar pathway.- - - - - - - - - - ranking = 1164.0206247967keywords = supratentorial (Clic here for more details about this article) |
3/9. Diagnostic difficulties in childhood bilateral thalamic astrocytomas.We report on two children with bilateral thalamic astrocytomas. The first patient developed psychomotor regression at the age of 20 months followed by rapidly progressive ataxia, intention tremor, slurred speech, and bouts of drowsiness. magnetic resonance imaging (MRI) of the brain showed swelling and high signal intensity in both thalami accompanied by supratentorial hydrocephalus. The second patient presented with progressive cerebellar ataxia, headache, and vomiting at the age of 11 years. MRI of the brain revealed symmetrical, hyperintense and sharply delineated swelling of both thalami. Additional lesions were seen in the cerebellum and the right temporal lobe. In both cases proton magnetic resonance spectroscopy (MRS) of the lesions showed a striking decrease of the neuronal marker N-acetylaspartate, an increase of choline-containing compounds, and a minimal lactate peak. Stereotactic biopsies from the thalamus of the first patient and from a cerebellar lesion of the second patient finally revealed glial tumors, namely a diffuse astrocytoma of world health organization (WHO) grade II in the first patient and an anaplastic astrocytoma of WHO grade III in the second patient. We conclude that the clinical manifestations and MRI patterns of bilateral thalamic astrocytomas are very similar to those of encephalitis and neurometabolic disorders and should therefore be included in the differential diagnosis of these encephalopathies.- - - - - - - - - - ranking = 1164.0206247967keywords = supratentorial (Clic here for more details about this article) |
4/9. Atypical spasmus nutans as an initial sign of thalamic neoplasm.A patient is described who presented with dissociated nystagmus (atypical spasmus nutans) and an underlying pulvinar-tectal lesion. Atypical spasmus nutans is discussed and clinicians are alerted to a spectrum of possible etiologies.- - - - - - - - - - ranking = 4keywords = neoplasm (Clic here for more details about this article) |
5/9. Familial neurofibromatosis 1 with germinoma involving the basal ganglion and thalamus.Intracranial germinoma associated with neurofibromatosis 1 (NF-1) has never been documented previously. We report a case of familial NF-1 with a germinoma involving the right basal ganglion and thalamus. A 12-year-old boy presented with multiple cafe-au-lait spots and a family history of neurofibromatosis in his mother, one of two siblings, and his maternal grandfather. His intracranial lesion was subtotally resected. Histologically, it was a pure germinoma. serum alpha-feto protein and beta-human chorionic gonadotropin levels were within the normal range. Postoperative myelographic examination and cerebrospinal fluid cytology study showed no evidence of subarachnoid seeding. The patient received postoperative combination chemotherapy resulting in complete response and clearance of the residual tumor. Although this finding of an intracranial germinoma in a patient with familial NF-1 may be coincident, it is suggestive of a potential genetic predisposition. Longitudinal evaluation for the possibility of neoplasm, especially germ cell tumor, in basal ganglion lesions in NF-1 patients is necessary.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/9. Isolated hemiataxia after supratentorial brain infarction.Acute isolated hemiataxia is in most cases due to infratentorial (cerebellar) stroke. It has only twice been described in supratentorial stroke--namely, after thalamic infarction and a capsular haemorrhage. Three patients with isolated hemiataxia after a supratentorial brain infarct are described. These patients were seen in a period of five years during which 899 patients with a first supratentorial brain infarct were registered. Clinically the hemiataxia was of the cerebellar type. In two patients, CT and MRI showed a small, deep (lacunar) infarct restricted to the posterior limb of the internal capsule, a site not previously reported in isolated hemiataxia. The third patient had a small, deep (lacunar) infarct in the thalamus extending into the adjacent posterior limb of the internal capsule. Isolated hemiataxia after a supratentorial brain infarct is a very rare clinical stroke syndrome. The cerebellar type hemiataxia was most likely caused by interruption of the cerebellar pathways at the level of the internal capsule. Our cases confirm prior observations that the cerebellar pathways run through the posterior part of the posterior limb of the internal capsule separately from the motor and sensory pathways.- - - - - - - - - - ranking = 9312.1649983733keywords = supratentorial (Clic here for more details about this article) |
7/9. Contralateral conjugate eye deviation in acute supratentorial lesions.BACKGROUND: Conjugate eye deviation (CED) in patients with acute supratentorial lesions is generally directed ipsilateral to the lesioned hemisphere. Incidentally, CED occurs to the contralateral side. We report five new cases and review previously published reports to elucidate the lesion responsible for and the mechanism underlying this phenomenon. CASE DESCRIPTIONS: In a prospective study of 133 consecutive patients with CED caused by an acute supratentorial lesion, 5 patients showed contralateral CED. This was caused by an intracerebral hemorrhage located thalamic (n = 2), frontoparietal (n = 1), and frontoparietotemporal (n = 1). In 1 patient the cause was a subdural hematoma, an association that has not been reported earlier. Four of the 5 patients died. All patients had clinical signs of rostral brain stem dysfunction and a shift of midline structures on computed tomographic scan or at autopsy. CONCLUSIONS: Contralateral CED is always associated with hemorrhagic lesions, most commonly in the thalamus. The prognosis of patients with this sign is generally poor. Involvement of descending oculomotor pathways from the contralateral hemisphere at midbrain level is the most probable explanation for this phenomenon.- - - - - - - - - - ranking = 6984.12374878keywords = supratentorial (Clic here for more details about this article) |
8/9. Parainfectious optic neuritis and encephalomyelitis. A report of two cases with thalamic involvement.Two children developed mental status alteration and bilateral profound visual loss secondary to optic neuritis. The clinical picture was consistent with parainfectious encephalomyelitis. magnetic resonance imaging showed bilateral involvement of the thalamus in both cases. In one case the thalamic involvement was solitary and was suspected initially to represent a primary thalamic neoplasm. This was ruled out by a stereotactic biopsy of the thalamus, which showed perivascular inflammation consistent with parainfectious encephalomyelitis. The clinical and neuroimaging findings improved significantly following corticosteroid administration. Several relapses occurred upon initial attempts at corticosteroid cessation.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/9. Pleomorphic xanthoastrocytoma: clinical, imaging and pathological features of four cases.Four cases of pleomorphic xanthoastrocytoma (PXA) were collected from among 688 glioma patients who underwent operation at the Institute of neurosurgery, University of Naples "Federico II" between January 1973 and December 1994. Three were females and one male, ranging in age from 10 months to 65 years. Three tumors were superficial in location, appearing as a meningo-cerebral mass in the temporo-parietal region. In one case, the tumor was situated deep within the brain (capsulo-thalamic region), without contact with leptomeninges. Three patients had experienced epileptic seizures, whereas one patient presented with an ictal episode. Tumor excision was grossly total in two cases, and subtotal in the remaining two. In three cases, histological examination demonstrated a "typical" PXA; conversely one tumor (subtotal excised) was an "atypical" PXA. The two patients with incomplete surgical resection were postoperatively treated with fractionated brain radiation therapy. Of the two patients who had grossly total removals, one showed tumor recurrence 6 years after surgery, and underwent operation (the recurrent neoplasm did not exhibit malignant transformation); the second patient was free of tumor at 14 months following craniotomy. Of the two patients who had undergone subtotal removals, one died because of massive regrowth of the lesion 22 months after surgery, whereas the second patient was asymptomatic at 1 year follow up.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |