Lumbo-sacral malformations and spina bifida occulta in medieval skeletons from Brittany.

INTRODUCTION: Compared with the other French regions, the incidence of neural tube defects is raised in Brittany. It can be explained by the Celtic origin of the Britton people, who migrated from Great Britain in the High Middle Ages. Notwithstanding, there are no historical or archeological evidences of the occurrence of these pathological conditions in medieval Brittany. MATERIALS AND METHODS: We investigated the incidence of lumbo-sacral malformations on the skeletal remains of 30 individuals excavated from the necropolis of Saint-Urnel (southwest Brittany). RESULTS: We found out several anatomical variations among five specimens, three of which had spinal dysraphism involving the sacrum. CONCLUSION: Our results enrich the very few paleopathological data about spinal dysraphism, from the Hippocratic Corpus to the first description of Spina Bifida in sixteenth century. But, their interpretation remains delicate until the same genetic factors are shown in the etiology of both open and closed spinal dysraphism.

Theoretical considerations on the pathophysiology of normal pressure hydrocephalus (NPH) and NPH-related dementia.

Normal pressure hydrocephalus (NPH) is considered to be an example of reversible dementia although clinical improvement after shunting varies from subject to subject, and recent studies have pointed to a possible link with other dementia. The authors consider that the craniospinal compartment is a partially closed sphere with control device systems represented by the spinal axis and the sagittal sinus-arachnoid villi complex which interact with each other in the clinical patient setting. We hypothesise that changing spinal compliance by altering the flow process and CSF dynamics lead to hydrocephalus. Therefore four NPH types have been distinguished according to the alterations in spinal compliance, decrease in CSF absorption at the sagittal sinus or both occurrences. The authors consider that NPH and NPH-related diseases (NPH-RD) are initiated by the same common final pathway and demonstrate that NPH could represent an initial stage of NPH-RD. Progression of clinical signs can be explained as damage to the cerebral tissue by both intermittent increased intracranial pressure and pulse pressure waves leading to periventricular ischaemia. In addition, they believe that both volume equilibrium and spinal compliance are restored in patients who improve after CSF shunt, whereas in patients whose condition does not improve, only volume equilibrium is restored and not spinal compliance, which was the underlying cause of hydrocephalus in such cases. They therefore wonder whether cervical decompression should not be indicated in patients who show no improvement. Although attractive, this analysis warrants confirmation from clinical, radiological, and hydrodynamic studies.

Idiopathic normal pressure hydrocephalus: theoretical concept of a spinal etiology.

Normal pressure hydrocephalus (NPH) is an adult syndrome characterised by a combination of gait disturbance, varying degrees of cognitive decline, urinary incontinence, ventricular enlargement and normal mean intracranial pressure. Since this syndrome was first described, its pathophysiology has been a matter of great debate, although it is now considered that NPH could be divided into two groups: cases with unknown etiology (idiopathic normal pressure hydrocephalus, or INPH) and those which develop from several known causes (such as trauma, meningitis or subarachnoid haemorrhage). The pathophysiology of INPH is still unclear and a matter of debate. In this manuscript, the current pathophysiological conditions of INPH are analysed and the authors put forward the theory that the disease is a dynamic syndrome which occurs in patients who have suffered a significant loss of spinal compliance over time. Consequently, intracranial pressure increases more during systole in INPH patients because it cannot be compensated for by the escape of CSF into the spinal canal as effectively, due to the reduced volume or lack of distension of the spinal canal. This leads to an increase in ventricular size and causes cumulative brain damage over a long period of time and accounts for the slow, progressive nature of NPH. The loss of spinal compliance with age is fundamental to the proposed theory which provides a theoretical justification for studying the spinal canal in INPH and investigating the relationship between the progressive narrowing of the spinal canal and the compensating ability of the craniospinal system.