Cerebellar involvement in multifocal eosinophilic granuloma: Demonstration by computerized tomographic scanning

Adornato, Bruce T.; Eil, Charles; Head, Gordon L.; Loriaux, D. Lynn

doi:10.1002/ana.410070206

Cited by 28 publications

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“…Two patients showed a deterioration of a prediagnosed myopathy 4 6 whereas one woman experienced the manifestation of a lipid storage myopathy with decreased concentrations of muscle and plasma carnitine after delivery. 7 The muscle weakness started some weeks after delivery and reached a peak within several months (table). Two other patients with mitochondrial myopathies (one with an unidentified enzyme defect, 8 one with carnitine palmitoyl transferase deficiency 9 ) were asymptomatic during pregnancy and after delivery.…”

Section: Spontaneous Recovery Of Opsoclonusmyoclonus Syndrome Caused mentioning

confidence: 99%

“…5 Unlike our patient, however, his brain MRI disclosed multiple white matter enhanced lesions in both cerebral hemispheres, pons, medulla, and brachium pontis. Furthermore, some cases with another form of histiocytosismultifocal eosinophilic granuloma (MEG)-were reported to have presented as progressive cerebellar syndrome, [6][7][8] and we previously reported a case with MEG showing progressive cerebellar and brainstem symptoms and multiple enhanced lesions on MRI.…”

Section: Botulinum Toxin Type a In The Treatment Of Upper Limb Spastimentioning

confidence: 99%

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Minimally Invasive Therapy of The Brain

Hardy¹

1998

Journal of Neurology, Neurosurgery & Psychiatry

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Spontaneous recovery of opsoclonusmyoclonus syndrome caused by enterovirus infectionOpsoclonus-myoclonus syndrome is a well recognised disease in children. The aetiology, except for neuroblastoma accounting for a few patients, remains unknown.1 Viral infection is one of the presumed physiopathological mechanisms. We report a case of opsoclonus-myoclonus syndrome due to enterovirus infection with spontaneous remission, no recurrence, and full recovery after 36 months follow up.A two year old girl, with a previously normal development, exhibited acute ataxia after a two day history of fever and vomiting. One day later, she developed myoclonic jerks and opsoclonus. Initially, she was febrile (39°C), irritable, and unable to sit or to stand because of truncal ataxia. There was resistance to neck flexion. Rapid irregular and oscillatory eye movements were seen with continual myoclonus of the neck and limbs. Enterovirus RNA was detected in CSF at day 3 by polymerase chain reaction (PCR). 2 The CSF, on day 3, contained 113 leucocytes/ mm 3 (98% lymphocytes and 2% polymorphonuclear leucocytes), 25 mg/dl protein, and normal glucose concentrations. The interferon-titre in CSF was raised (3 U/Ml). At day 15, the CSF contained 20 leucocytes/ mm 3 . The interferon-titre was normal in both CSF and blood. EEG and brain MRI were normal. A chest radiograph, abdominal ultrasound examination, and urine titres of homovanillic acid and vanillylmandelic acid were normal. Viral culture from CSF was negative. Treatment with clobazam (1mg/kg/ day) led to resolution of the myoclonus over 15 days. Developmental and neurological examinations were normal 36 months later (Denver developmental screening test).We report a child with opsoclonusmyoclonus syndrome associated with enterovirus infection. Enterovirus infection associated with this syndrome has been rarely reported but could be underestimated. 3 In fact, a diagnostic method with a high sensitivity was only recently provided by PCR, whereas the previous diagnostic testnamely, viral culture and serological assay, had a low diagnosis yield. Development of enterovirus RNA detection by PCR in blood and CSF should be more systematically performed in opsoclonus-myoclonus syndrome to better define the place of this infection as a cause of this syndrome.The relation between enterovirus infection and opsoclonus myoclonus syndrome could have some therapeutic implications. Interestingly, this patient and the two other similar cases reported in the literature had a spontaneous recovery in less than 15 days. 3 Despite the lack of a well designed therapeutic trial, corticosteroid treatment is commonly used in patients with opsoclonus-myoclonus syndrome whatever the aetiology. Most of the patients given this treatment have shown a resolution of ataxic and myoclonic symptoms. However, long term follow up shows nearly constant mental sequelae and a high rate of myoclonic relapse.1 There is clear experimental evidence Neurol Neurosurg Psychiatry 1998;64:406-422 406 that corticosteroids potentiat...

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Section: Spontaneous Recovery Of Opsoclonusmyoclonus Syndrome Caused mentioning

confidence: 99%

Section: Botulinum Toxin Type a In The Treatment Of Upper Limb Spastimentioning

confidence: 99%

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Minimally Invasive Therapy of The Brain

Hardy¹

1998

Journal of Neurology, Neurosurgery & Psychiatry

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Idiopathic Central Diabetes Insipidus Followed by Progressive Spastic Cerebral Ataxia

Birnbaum¹

1989

Arch Neurol

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Multifocal eosinophilic granuloma and the cerebellum

Thorpy

Crosley

1980

Annals of Neurology

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AdQmatq et al [I] reported seeing hypodense areaz-Ln the cerebellum on computed t o m o e h y (CT) of the brain in two patients with multifocal eosino~hilic granuloma. We have cared for two patients with similar lesions whose findings help answer some of the questions raised by Adornato and his colleagues.A boy developed proptosis at 2 years of age. Biopsy of an orbital mass revealed eosinophilic granuloma, and chemotherapy with vincristine and prednisone was begun. Six months later he developed gait ataxia. A C T scan showed symmetrical hypodense areas in the cerebellum in the region of the dentate nuclei. Hounsfield numbers in the lesions were between 9 and 31. The orbital mass persisted during this period, and radiation therapy with 1,000 rads was administered to the left frontal and orbital areas. C T scans repeated over the next two years showed no changes in the cerebellar lesions.A ll/z-year-old boy was found by biopsy to have an eosinophilic granuloma of the left orbit. He developed multiple skull lesions and received 4,800 rads of radiation therapy and chemotherapy over the next three years; the radiation ports were restricted to the orbits and temporal areas. A progressive truncal ataxia occurred, and a C T scan showed bilateral hypodense areas in the cerebellum in the region of the dentate nuclei. A course of radiation therapy was administered to the posterior fossa. Over the next three years, CT scans showed no progression of the hypodense areas. The ataxia has steadily progressed.Cerebellar disturbance in multifocal eosinophilic granuloma has been associated with dentate calcification and atrophy of the cerebellum [ 11, xanthomatous granulomas [ 5 ] , and demyelinating lesions 12, 31 with or without concomitant xanthomatous infiltration. Adornato et al [ 13 suggested that the hypodense cerebellar lesions in their patients were unrelated to prior radiation therapy. The presence of the cerebellar lesions in our first patient preceded irradiation, and the radiation therapy administered to our second patient did not include the posterior fossa until after the cerebellar lesions were demonstrated. Solely granulomatous infiltration also seems an unlikely explanation for the lesions, as histologically proved areas of granulomatous tissue have been shown to be isodense or hyperdense on C T scan [ 5 ] . The orbital lesions in our patients were of increased density. Adornato and his associates concluded that the areas of hypodensity in their patients were regions of "demyelination coexistent with histiocytic infiltration" [ 1 I. We also favor demyelination but think it may occur without coexistent infiltration of histiocytes [2, 31. Subsequent to the discovery of cerebellar lesions in our patients there has been no recurrence of multifocal eosinophilic granuloma; the cerebellar symptoms have persisted or progressed, but the lesions have remained unchanged on CT scan.The wisdom of treating such lesions with radiation therapy must also be questioned. Chemotherapy and irradiation in leukemic children have produc...

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Cerebellar involvement in multifocal eosinophilic granuloma: Demonstration by computerized tomographic scanning

Cited by 28 publications

References 11 publications

Minimally Invasive Therapy of The Brain

Minimally Invasive Therapy of The Brain

Idiopathic Central Diabetes Insipidus Followed by Progressive Spastic Cerebral Ataxia

Multifocal eosinophilic granuloma and the cerebellum

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