DRUG-RESISTANT EPILEPSY IN CASE OF CRANIOCEREBRAL DISPROPORTION. A PILOT STUDY

The problem of ineff ective drug therapy of epilepsy continues to be relevant during many decades and determines many key research trends in clinical and fundamental epileptology. Despite the emergence of a large number of various antiepileptic drugs the eff ectiveness of drug therapy for epilepsy has remained almost unchanged over the past decades. In this article drug-resistance in epilepsy is considered as a consequence of a concomitant pathological process in the form of craniocerebral disproportion.

Shunt Overdrainage: Reappraisal of the Syndrome and Proposal for an Integrative Model

Although shunt overdrainage is a well-known complication in hydrocephalus management, the problem has been underestimated. Current literature suggests that the topic requires more examination. An insight into this condition is limited by a lack of universally agreed-upon diagnostic criteria, heterogeneity of published series, the multitude of different management options and misunderstanding of relationships among pathophysiological mechanisms involved. We carried out a review of the literature on clinical, radiological, intracranial pressure (ICP), pathophysiological and treatment concepts to finally propose an integrative model. Active prophylaxis and management are proposed according to this model based on determination of pathophysiological mechanisms and predisposing factors behind each individual case. As pathophysiology is progressively multifactorial, prevention of siphoning with gravitational valves or antisiphon devices is mandatory to avoid or minimize further complications. Shunt optimization or transferal and neuroendoscopy may be recommended when ventricular collapse and cerebrospinal fluid isolation appear. Cranial expansion may be useful in congenital or acquired craniocerebral disproportion and shunting the subarachnoid space in communicating venous hydrocephalus and idiopathic intracranial hypertension.

Successful treatment of post-shunt craniocerebral disproportion by coupling gradual external cranial vault distraction with continuous intracranial pressure monitoring

A subset of hydrocephalic patients in whom shunts are placed at an early age will develop craniocerebral disproportion (CCD), an iatrogenic mismatch between the fixed intracranial volume and the growing brain. The lack of a reliable, reproducible method to diagnose this condition, however, has hampered attempts to treat it appropriately. For those practitioners who acknowledge the need to create more intracranial space in these patients, the lack of agreed-upon therapeutic end points for cranial vault expansion has limited the use of such techniques and has sometimes led to problems of underexpansion. Here, the authors present a definition of CCD based primarily on the temporal correlation of plateau waves on intracranial pressure (ICP) monitoring and headache exacerbation. The authors describe a technique of exploiting continued ICP monitoring during progressive cranial expansion in which the goal of distraction is the cessation of plateau waves. Previously encountered problems of underexpansion may be mitigated through the simultaneous use of ICP monitors and gradual cranial expansion over time.

Globular subdural hematoma in a shunt-treated infant

Subdural hematoma (SDH) is a well-known sequela of ventriculoperitoneal shunt insertion for hydrocephalus, usually spreads out over the cerebral convexity, and appears as a crescent-shaped lesion on imaging. The authors report the rare case of an infant with a globular SDH, which MR imaging revealed as a round mass lesion. A 13-month-old girl with a history of severe congenital hydrocephalus associated with myeloschisis underwent ventriculoperitoneal shunt placement after repair of myeloschisis and developed convulsive seizure. Magnetic resonance imaging showed a large subdural round mass with a lesion like a pedestal in the left parietal region, suggesting a globular SDH surrounded by a thin cerebral mantle. Because of the seizures and because the hematoma did not respond to a change in valve pressure, the patient underwent a craniotomy to remove the hematoma and to resect the thick outer membranes of multiple layers. Postoperative MR imaging demonstrated the disappearance of the SDH, and no additional shunt complication was observed during a long follow-up period. To the authors' knowledge, this is the first report of a patient with globular SDH published in the literature. The authors postulate that the globular SDH was caused by the thin cerebral mantle associated with severe craniocerebral disproportion, and they discuss the possible mechanisms for this unique formation.