gms | German Medical Science

Cavernous haemangiomas (CH) consist of venous vascular malformations which occur in 0.4% of the population. CH represent 5-13% of all vascular malformations. Usually CH patients present with seizures, rarely with haemorrhage. CH can be sporadic or familial, single or multiple (usually not more than 3 lesions). Multiple cavernous angiomas are more frequent in the familial type.

The case of a 67-year-old man is reported here with more than 100 cavernous malformations in the brain including the brainstem and none in the spinal cord. Examination revealed multiple seborrhoeic keratoses and angiomas on the upper part of the body as well as multiple angiomas in the face. MR images showed unilateral acoustic neuroma on the right side.

This patient presented a 40y history of complex focal epilepsy. In 1987 a cranial computertomography (CCT) was performed and 5 supratentorial, partly calcified lesions were detected. At that time, the diagnosis granulomatosis disease of unknown origin was considered. Since 2004 the patient had increasing problems with walking because of dizziness and an uncertain feeling. The CCT from August 2004 showed an increase in the incidence and size of these partly calcified lesions. In September 2004 one lesion was biopsied using stereotaxy (histological diagnosis: vascular lesion). The MRI showed more than 100 cavernous haemangiomas, located in the supra- and infratentorial space as well as a unilateral acoustic neuroma on the right side. Whole body MRI and MRI of the spinal cord showed multiple haemangiomas of the cervical and thoracic vertebral bodies and excluded other vascular malformations. Clinically the patient presented with complex focal epilepsy, gait disturbance, progressive dementia, unilateral tinnitus and hypacusis (right side).

Central nervous system cavernomatosis with more than 100 lesions is uncommon. To our knowledge only one case has been reported so far with more than 100 central nervous system cavernous malformations, including several intramedullar spinal cord lesions. We reported an interesting and uncommon case with multiple CH, especially in combination with multiple haemangiomas of the cervical and thoracic vertebral bodies, multiple skin lesions and a unilateral acoustic neuroma.