Biomedical subjects
Publications and source records attributed to P Demaerel.
A girl of eight years of age is described with the Hallervorden-Spatz syndrome. Optic atrophy with progressive visual loss was the only presenting symptom for three years. Behavioural and motor disturbances emerged after that time. Optic atrophy can be the first and for some time only symptom of the Hallervorden-Spatz syndrome.
Two cases of non-invasive aspergillosis of the nose and paranasal sinuses are described. The first presented with left proptosis and ophthalmoplegia. Imaging and histology showed a maxillary sinus aspergilloma. The second case presented as a compressive optic neuropathy and histology showed allergic aspergillus sinusitis. The pathological distinction between invasive and non-invasive forms of aspergillus sinusitis is important as in invasive aspergillosis surgical treatment is most effectively combined with systemic antifungal treatment, whereas in aspergilloma of the paranasal sinuses surgical drainage of the sinuses alone is usually sufficient, and in allergic aspergillus sinusitis surgery is best combined with systemic or topical steroids. The distinction between invasive and non-invasive forms is particularly important as both may present with cranial neuropathies.
The authors present three patients with the diagnosis of septo-optic dysplasia or 'de Morsier' syndrome. They stress the important role of the ophthalmologist in the diagnosis. With the advent of MRI, subtle central nervous system abnormalities have been recognized. A close ophthalmological and endocrinological follow-up is necessary.
MR images of 17 patients with spondylitis were reviewed. T1 weighted spin-echo scans were obtained in 17 patients and proton density and T2 weighted scans were obtained in 15 patients. Unenhanced and gadopentetate dimeglumine enhanced scans were obtained in all patients. Five patients had pyogenic spondylitis, two patients tuberculous spondylitis, two patients fungal spondylitis and eight patients postoperative spondylitis. The four criteria described by Thrush and Enzmann were generally applicable in our study: 1) narrowing of the intervertebral disc; 2) cortical bone erosion; 3) abnormal signal in the adjacent vertebral bodies and 4) abnormal paraspinous or epidural soft tissue. In addition to plain sagittal T1 and T2 weighted images we suggest the routine administration of gadopentetate dimeglumine to assess the extent of the soft tissue mass and to differentiate postoperative spondylitis from a normal postoperative course, by showing disc enhancement. Disc enhancement occurs infrequently in the normal postoperative course. If it is associated with adjacent vertebral bone marrow changes it should be considered as postoperative spondylitis. A differential diagnosis between pyogenic, tuberculous, fungal and postoperative spondylitis was not possible although the pattern of enhancement in tuberculous spondylitis was different from the other cases of spondylitis.
We present a case of spinal epidermoid tumour that was probably caused by lumbar punctures. The tumour was detected on MRI in a child with tuberous sclerosis complaining of atypical low back pain. Preoperatively the tumour was considered to be a cystic astrocytoma of ependymoma of the conus terminalis. The association of tuberous sclerosis with a spinal tumour is rare but has been recorded in the literature. The diagnosis was an epidermoid tumour (E.T.). The MRI appearances were unusual for a spinal ET: the signal intensity changes were different from those reported in the literature and the presence of calcification and cavitation has not yet been reported. This case represents a diagnostic pitfall, since both entities were unrelated.
PURPOSE: To study the clinical and radiologic findings in 15 patients presenting with both developmental venous anomalies and cavernous angiomas and to determine which lesions caused the acute clinical symptoms. METHODS: Lesions were infratentorial in 7 patients and supratentorial in 7 other patients. In one patient the developmental venous anomaly was infratentorial and the cavernous angioma supratentorial. In all 15 patients clinical examination and CT were performed. In 10 patients MR was performed at the time of the initial CT scan. In 5 patients only late MR is available. RESULTS: Acute clinical signs were present in 9 patients (epilepsy, hemiplegia, meningeal signs, cranial nerve deficit). On CT the cavernous angioma was missed in 9 patients, in 6 patients because of acute hemorrhage. On MR the cavernous angioma was missed in 1 of 2 cases with acute hemorrhage but seen in all other patients. On repeat MR all cavernous angiomas were correctly identified. On CT, 11 developmental venous anomalies were seen. On MR all developmental venous anomalies were seen. CONCLUSIONS: The frequent association of developmental venous anomalies and cavernous angiomas is confirmed. MR is superior to CT in the detection of both cavernous angiomas and developmental venous anomalies. Furthermore our data support the hypothesis that cavernous angiomas and not the developmental venous anomalies cause the acute clinical symptoms because of hemorrhage.
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We report the MR appearance of a rare case of Lyme disease presenting as diffuse leptomeningeal enhancement in the absence of parenchymal lesions. In the appropriate clinical setting, one should consider Lyme disease in the differential diagnosis of meningeal enhancement.
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CT findings on 170 patients presenting with isolated moderate to severe neurodevelopmental delay have been compared with the final diagnosis. MRI was undertaken in 29 patients. Eighty per cent of the patients remained undiagnosed, and although the MRI findings were abnormal in 65.5% compared with only 30% of the CT examinations, imaging uncommonly suggested a specific diagnosis. Biochemical and chromosomal investigations were significantly more diagnostic. The results of these studies should restrict the number of non-contributory neuroradiological examinations. The superior intrinsic contrast of MRI will detect more lesions, particularly in white matter, but these are rarely diagnostic and where access to MRI is limited, CT is usually adequate.
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Recent reports indicate that fungal sinusitis is more common than previously thought. Four different histological subtypes of Aspergillus sinusitis are now recognized. Fulminant Aspergillosis is usually seen in immunosuppressed patients, and chronic invasive Aspergillus sinusitis, allergic Aspergillus sinusitis and Aspergilloma are seen in the non-immunocompromised individual. We present a case of progressive unilateral visual loss in a 30-year-old woman. The unusual magnetic resonance findings and the value of computed tomography are discussed.
Cerebral metastases of malignant melanoma usually have a characteristic appearance on CT and MRI. They present as multiple small lesions at the junction of grey and white matter. On CT the lesions are slightly hyperdense with moderate contrast enhancement. On MRI, typical T1 and T2 shortening is seen. In our case these findings were due to abundant presence of melanin without associated hemorrhage.
A case of intracerebral primitive neuro-ectodermal tumor (PNET) is presented. PNETs are rare, undifferentiated neoplasms occurring primarily in children and young adults. These tumors exhibit a highly malignant behaviour with a tendency to disseminate along the cerebrospinal fluid (CSF) pathways. Preoperative diagnosis is difficult, owing to the non-specific CT and MR appearances and to the rarity of this tumor. Our patient was 33 years old, which is uncommon. CT findings initially suggested an intracerebral hematoma. Later on, CT and MR findings were specific for a brain tumor and showed a solid, non-calcified invasive mass, with poor perilesional edema.
The CT and MRI appearances of 5 patients with Cockayne's syndrome, 5 with ataxia telangiectasia and 1 with Fanconi's anaemia are reported. These conditions, together with Bloom's syndrome and xeroderma pigmentosum are regarded as disorders of DNA repair. Characteristic CT and MRI features of Cockayne's syndrome include generalised atrophy, calcification in basal ganglia and dentate nuclei and white matter low density. Neuroradiological findings in the other DNA repair disorders are nonspecific.
The association of an acoustic neuroma with an ipsilateral venous anomaly or angioma is reported in two patients. In the first, the venous angioma was situated low in the posterior fossa, and was of no significance surgically. In the second, the angioma was strongly attached to the capsule of the tumour. On MRI and angiography the lesion appeared as a large draining vein, encircling the tumour. At operation, extensive dissection of the angioma from the neuroma appeared necessary to prevent venous infarction of the brain stem.
In a rare case of teratocarcinoma of the pineal gland, MRI accurately depicted the primary tumor and diffuse enhancing drop metastases along the surface of the brain stem and the spinal cord.
The magnetic resonance imaging (MRI) findings in eight patients with herpes simplex meningoencephalitis were reviewed: 14 examinations were analysed. The most striking finding was high signal intensity in the temporal lobe(s) with the typical configuration known from CT. Meningeal enhancement after Gd-DTPA administration was clearly seen in four patients. Haemorrhagic changes are much better seen on MRI than on CT. When adequate motion control can be achieved, MRI becomes the examination of choice in the diagnosis and follow-up of herpes simplex encephalitis. Localized 1H MR spectroscopy also proved promising in the study of neuronal loss.