Outcome of palliative embolization of bone metastases in differentiated thyroid carcinoma.

We investigated the effects of selective embolization in patients with symptomatic bone metastases of differentiated thyroid carcinoma. A total of 41 embolizations was performed in 16 patients. We studied the follow-up (range, 2 months to 8.6 yr) after the first embolization by evaluation of clinical symptoms and tumor dimensions. Success was defined as an improvement in clinical symptoms without tumor progression. The procedure was successful in 24 of 41 occasions (59%). Twenty-six embolizations were preceded or followed up by additional therapies, consisting of surgery (laminectomy), external irradiation, or radioiodine. Subgroup analysis revealed that these additional therapies did not influence the success rate; however, a potential effect on success duration may be present: for embolizations without additional radioiodine or external irradiation therapy, the median success duration was 6.5 months; for embolizations combined with additional radioiodine or external irradiation, this was 15 months (P = 0.0146). The ultimate outcome of the patients was unfavorable: nine patients died and five patients have progressive disease. We concluded that selective embolization of bone metastases may be considered a palliative therapy that may induce rapid, but transient, relief of symptoms. Combination with radioiodine or external irradiation may prolong the duration of success.

[Embolization of skeletal metastases in patients with differentiated thyroid carcinoma]. / Embolisatie van skeletmetastasen bij patiënten met gedifferentieerd schildkliercarcinoom.

Treatment options in patients with bone metastases of differentiated thyroid carcinoma are limited and mostly aimed at palliation. Conventional treatment modalities are: radioiodine therapy, surgery or external irradiation. A lesser known option is selective embolization of tumour metastases. During selective catheterization of the arteries that feed the metastases embolization material (e.g. polyvinyl alcohol particles) is injected into the local vasculature under radiographic control. The embolization is immediately evaluated angiographically. This therapy was used in three patients with differentiated thyroid carcinoma, a 60-year-old man suffering from back ache, paresis and afterwards paralysis of the lower body parts with incontinence, and in two women aged 59 years (suffering from diplopia and a pelvic metastasis) and aged 27 years (suffering from neurological symptoms or pain of the right leg due to a pelvic metastasis). In all three the symptoms disappeared after the embolization and ensuing treatment with radioactive iodine. Due to recurrent increase in serum thyroglobulin concentration repeated re-embolization was necessary, but during a follow-up period of 3-5 years the palliation remained adequate. Selective embolization appears to be a safe and efficacious treatment, with good tolerability for the patient. Embolization alone or preferably preceded by radioiodine therapy may lead to decreased tumour progression and often gives rise to immediate relief of symptoms.

Embolization for vertebral metastases of follicular thyroid carcinoma.

The technique of selective embolization has been applied for years in the treatment of vascular anomalies, severe hemorrhage and benign or malignant tumors, notably vertebral metastases of renal cell carcinoma. Because this technique is relatively easy to perform and offers immediate relief of symptoms, it is an attractive option for patients with vertebral metastases of thyroid carcinoma with signs of spinal cord compression. In these patients, other treatment modalities like radioactive iodine, external irradiation, or surgery are more cumbersome or less effective in the short term. We describe four patients with metastasized follicular thyroid carcinoma, presenting with neurological symptoms due to vertebral metastases. All patients had undergone total thyroidectomy, ranging from 1 month to 4 yr before embolization. Embolization was combined with iodine-131 therapy when appropriate. Selective catheterization of the arteries feeding the metastases was performed, followed by infusion of polyvinyl alcohol particles (Ivalon). The procedure was technically successful in all patients without adverse effects. In the patients described, embolization resulted in rapid resolution of neurological symptoms, sometimes within hours. The therapeutic effect lasted from months to years. We conclude that embolization of vertebral metastases of follicular thyroid carcinoma is an attractive palliative therapeutic option that may offer rapid relief of symptoms.

Bilateral sensorineural deafness, partial agenesis of the corpus callosum, and arachnoid cysts in two sisters.

We describe two sisters (ages 10 and 3 years, respectively) with a normal development and a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, arachnoid cyst, and hydrocephalus. Neither girl has distinctive physical anomalies. In the oldest girl, there was a hearing loss of 80 dB bilaterally, and the most severe loss on audiogram was seen at 2,000-4,000 Hz. In the youngest girl, there was a hearing loss of 100 dB bilaterally. Above 2,000 Hz no neural reactions were seen. Cerebral magnetic resonance imaging in one girl and computed tomography in the other showed a partial agenesis of the corpus callosum and a cyst in the pineal region, causing an aqueduct stenosis by compression and consequent hydrocephalus. The parents have normal hearing, and brain magnetic resonance imaging showed no abnormalities. They are nonconsanguineous but from the same small village. This is the first report of a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, and an arachnoid cyst. The pattern of inheritance is probably autosomal recessive.

Three cases of isolated mucoceles and one case of an Aspergillus infection in the sphenoid sinus with orbital signs and symptoms.

Four patients with an isolated sphenoid lesion: three with a mucocele and one with an Aspergillus infection of the sphenoid sinus in whom the orbital impairment had a rhinogenic cause, are described. They all presented with one or more of the following features: visual loss, motility disturbances, visual field defects, and sensitivity loss of the infraorbital nerve. In one patient, optic disc edema was seen. Diagnostic imaging technologies such as computer tomography (CT) and magnetic resonance (MRI) make an early diagnosis possible but nevertheless, very large mucoceles with major functional loss were detected at a late stage of the disease. The Aspergillus infection was detected at an early stage without delay because of its specific appearance on CT and MRI. The signs and symptoms of these lesions are more striking in the orbital and neuro-ophthalmological field than in the ear-nose-throat (ENT) field, but nevertheless, primary treatment should be marsupialization by an ENT-surgeon.

Optic nerve glioma mimicking an optic nerve meningioma.

The case is described of an optic nerve glioma, mimicking an optic nerve meningioma in a man aged 41 years. CT, MRI and DSA revealed an enhancing tumor surrounding the optic nerve. Histopathologic examination of the removed optic nerve revealed a centrally located glioma surrounded by normal optic nerve fibres.

Percutaneous computed-tomography-guided thermocoagulation for osteoid osteomas.

Current treatment for osteoid osteomas is usually surgical excision of the nidus. We treated 18 patients with osteoid osteoma by percutaneous thermocoagulation of the nidus under computed-tomography guidance. The procedure was technically successful in all cases and there were no complications. Patients were discharged on the following morning and resumed normal activities immediately. All patients but one remained pain free during follow-up (range 3-15 months). A second thermocoagulation treatment relieved the recurrent symptoms in this patient. Percutaneous thermocoagulation appears to be effective for osteoid osteomas, and is a minimally invasive alternative to surgical resection.

Idiopathic isosexual central precocious puberty: magnetic resonance findings in 30 patients.

The purpose of this prospective study was to define the incidence of magnetic resonance imaging (MRI) abnormalities in the brain in patients with idiopathic central precocious puberty without any additional neurological signs and symptoms, and to evaluate the routine use of gadolinium contrast in these patients. 30 patients (29 girls, one boy; age range 1.9-11.9 years) with idiopathic central precocious puberty were studied. MRI of the brain in axial, coronal and sagittal planes was performed before and after administration of gadopentetate dimeglumine, with special attention to the region of the third ventricle. There are three major findings: (1) the height of the pituitary gland is increased up to adult size compared with normal individuals; (2) in four patients (13%) major structural abnormalities were found; three hamartomas of the tuber cinereum and one gliomatous process extending from the chiasm to the optic tract; and (3) the routine use of gadopentetate dimeglumine did not reveal new abnormalities although the lack of enhancement made a positive contribution to diagnostic certainty. We conclude that contrast enhanced MR examination is a safe and reliable method for the exclusion of abnormalities in children with precocious puberty and for the follow-up of those patients in whom abnormalities are present.

Neuroradiological investigations in cervical root avulsion.

Cervical myelography in combination with CT myelography is not fully reliable to demonstrate a partial or complete cervical root avulsion. MRI scanning can demonstrate large traumatic meningoceles or additional lesions, such as intramedullary or extradural haematomas, but not a root avulsion. In experimental conditions MR microscopy enables visualization of the avulsed root separated from the spinal cord. The anterior funiculus shows transverse tracts left behind by the avulsion at the original site of the motor fibres. However, the small bore of the current high field magnets and the very long acquisition time makes this method, as yet, unsuitable for application in man.

Magnetic resonance imaging of the so-called cerebral cryptic angiomas.

Cerebral cryptic angiomas are vascular malformations with variable histological appearance, but with similar radiological features. Angiography does not usually visualize the lesion. Computer tomography often detects the malformation, but frequently fails to make classify correctly. With magnetic resonance imaging (MRI) the cryptic angioma is studied best on T2 weighted images. The pattern of signal intensities differs both among and within the lesions. Evidence of small hemorrhages of different ages and flow phenomena are seen in all malformations. A total of 51 angiomas were demonstrated in 38 patients by MRI (excluding one patient with a very large number of angiomas). Enhanced or plain CT scans depicted respectively 16 and 6 lesions. In one case angiography was the only way to detect an angioma. Currently, MRI is the most sensitive method for the detection of cerebral cryptic angiomas.

Follow-up magnetic resonance imaging in Hallervorden-Spatz disease.

Bilateral high signal emitting areas in the globus pallidus surrounded by low signal emitting areas have been described as a typical MRI finding in Hallervorden-Spatz disease (HSD). We made a diagnosis of HSD in an 11-year-old girl with progressive dystonia of 4 years duration who showed these typical MRI abnormalities. An initial MRI at the age of 9 was normal. Pathological confirmation of these typical MRI findings has not yet been described, but earlier reports as well as our case suggest that MRI may be helpful in making a clinical diagnosis of HSD. This case further shows that MRI may be normal in an early stage of the disease.

Similar brain lesions in alcoholics and Korsakoff patients: MRI, psychometric and clinical findings.

MRI examination revealed similar brain lesions in 5 alcoholic Korsakoff patients and 5 chronic alcoholics without cognitive impairment. Not only cerebral atrophy and demyelination, but also lesions thought to be specific for the Wernicke-Korsakoff syndrome were equally prominent in both groups. The morphological abnormalities thought to be typical of Wernicke-Korsakoff syndrome are probably common features of chronic alcoholism and malnutrition. Marked atrophy of the operculae was found in all Korsakoff patients and in 3 out of 5 chronic alcoholics. Alcohol amnestic disorder may not exclusively result from diencephalic lesions, but also from temporal lesions.

Hereditary spastic dystonia: a new mitochondrial encephalopathy? Putaminal necrosis as a diagnostic sign.

A large kindred, in which either Leber's hereditary optic atrophy, or a hereditary spastic dystonia, or a combination of both manifested over many generations was restudied after the first report on it in 1964. NMR scans revealed bilateral, and, in two patients with hemidystonia, unilateral necrosis with shrinkage of the putamen, in one case associated with total disappearance of the head of the caudate nucleus. Except for age-appropriate cortical atrophy in one instance, no other changes were observed in the brain, brainstem, and cerebellum. The putaminal necrosis appears as typical "striatal slits" on the NMR scans. It is argued that this rare disease, since the princeps description in 1964 only reported in England (1986) and the U.S.A (1986), is most likely a singular type of mitochondrial encephalopathy: it is associated with Leber's optic atrophy, and the NMR changes observed have been signalled in other mitochondrial encephalomyelopathies, such as Leigh's disease and MELAS.

Lhermitte-Duclos disease and Cowden disease: a single phakomatosis.

Two unrelated patients with macrocephaly, seizures, and mild cerebellar signs had a dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). Both also had autosomal dominant Cowden disease as evidenced by facial, oral, and acral papules. In the two families, 9 sibs demonstrated the mucocutaneous lesions, thyroid disease, breast tumors, and ovarian tumors compatible with the diagnosis of Cowden disease. Some of the sibs also showed various degrees of neurological signs such as macrocephaly, mental retardation, seizures, tremor, and dysdiadochokinesia. Magnetic resonance imaging scans of sibs of one family demonstrated megalencephaly and other mild abnormalities. The occurrence of these two rare disorders in single patients is more than a coincidence, and the clinical findings in the combined condition establishes it as a new phakomatosis.

Sciatic neuritis as initial symptom of spontaneous clostridial myonecrosis.

A 21-year-old woman is reported with aplastic anaemia, who presented with pain in the leg. Rapid loss of sciatic nerve function followed. MRI showed irregular streaks of low intensity in the muscles of the pelvic region. A diagnosis of sciatic neuritis as initial symptom of clostridial myonecrosis was made.

Heredo-ataxia in a large Dutch pedigree. M.R.I. findings.

One segment of a large Dutch pedigree with heredo-ataxia is presented. The clinical and genetic features of the disease, together with the M.R.I. findings, indicate the diagnosis of autosomal dominant late onset olivo-ponto-cerebellar atrophy. The clinical picture remained invariant through successive generations. Emphasis is put on the diagnostic usefulness of M.R.I. technique in the absence of neuropathological data.