Varsik P

Neurocutaneous Syndromes: Developmental Dysplasias of the Early Period of Gestation. A Clinical and MRI Study

The neurocutaneous syndromes, also called phakomatoses or hamartomatous syndromes, are a highly variable and heterogenic group of dermatologic, somatic and nervous system disorders. Much attention is paid to these syndromes by neurologists because they are usually accompanied by epilepsy and mental retardation. Dermatological symptomatology is relatively variable, and somatic disturbances are very common, but nervous system disorders, both in clinical and in MRI pictures, are regularly observed. The normal process of neural crest formation in the 4th week of gestation is characterized by a period of rapid proliferation and differentiation of the neuroependymal cells and this process of neuroblast migration is often considered to be the most important developmental period of the brain. This process continues between the 10th and 22nd weeks of gestation and ends with the cellular apoptosis of 30–50% of participating neuroblasts. In some cases, the neuroblasts will not reach their predetermined site within t...

The Quest for the Cause of Spinal Cord Ischemic Lesions: Impaired Arterial Supply or Venous Drainage?

Incomplete transverse spinal cord lesion after ischemic insult of arterial origin is not uncommon, but ischemic spinal cord lesion caused by impairment of the venous drainage system is very rare. A 33-year-old man with the postinfective hypercoagulation syndrome and incomplete cervical spinal cord ischemic lesion with a typical clinical course as in Foix-Alajouanines syndrome is described. Postischemic and postnecrotic gliosis of the spinal cord tissue in segments C6-Th2 was confirmed at MRI examination. The location of the postischemic medullar lesion did not have the typical “pencil-like” appearance usually present in the impairment of arterial supply. Instead, the lesion had an atypical distribution of ischemic changes in the ventral marginal part of the spinal cord near the sulcomarginal incisura suggesting impairment of the venous drainage system. After low-molecular heparins and antibiotic treatment, the patient made a very good recovery with functional restitution. Spinal MR-angiography showed a normal arterial blood supply.

Latent Cerebral Venous Drainage Disorders: Etiopathogenesis, Clinical and Neuroradiodiagnostic Symptomatology

Impaired cerebral venous drainage and latent cerebral venous and sinuses thrombosis are common disorders. We describe five cases of consecutive latent disturbance of cerebral venous drainage and latent or oligosymptomatic cerebral venous and sinuses thrombosis (CVST) disclosing the need for prompt accurate etiological diagnosis. The risk of CVST in the presence of two or more etiological factors is increased. Very frequently the patients complaints and the clinical picture are oligosymptomatic and poor. In suspicion of impaired cerebral venous drainage, an examination of the cerebral vessels including phlebography is recommended. We assume that CT-Angiography, MR-Angiography or a classical DSA is inevitable in some cases for correct and successful treatment.