Die Camurati-Engelmannsche Erkrankung (progressive diaphysÄre Dysplasie). Differentialdiagnostische Probleme

Physicians should include CED in the differential diagnosis for patients with radiographic evidence of skull base thickening and synchronous cranial neuropathies or symptoms of elevated intracranial pressure. In mild forms of the disease, the clinical course of patients should be followed with serial examination, audiometric testing, and radiography. In select patients with progressive cranial base symptoms, aggressive wide decompression of involved neurovascular structures may provide benefit.

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Skull Base Manifestations of Camurati-Engelmann Disease

Carlson¹,

Beatty²,

Neff³

et al. 2010

Arch Otolaryngol Head Neck Surg

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Successful treatment with corticosteroid in a patient with progressive diaphyseal dysplasia

1995

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Camurati–Engelmann disease: imaging, clinical features and differential diagnosis

2009

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We report four sporadic and three familial patients with Camurati-Engelmann disease. One patient had follow-up examinations over 8 years. Pain in the extremities and muscle weakness were common clinical symptoms. Most patients also had cranial nerve impairment, hepatosplenomegaly, a waddling gait, and elevated serum alkaline phosphatase levels. Long bones were affected in all. We discuss the differential diagnosis for this interesting bone entity.

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Die Camurati-Engelmannsche Erkrankung (progressive diaphysÄre Dysplasie). Differentialdiagnostische Probleme

Cited by 3 publications

References 5 publications

Skull Base Manifestations of Camurati-Engelmann Disease

Skull Base Manifestations of Camurati-Engelmann Disease

Successful treatment with corticosteroid in a patient with progressive diaphyseal dysplasia

Camurati–Engelmann disease: imaging, clinical features and differential diagnosis

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