-
[show abstract]
[hide abstract]
ABSTRACT: The clinical features of Chiari I Malformation (CIM) may be related to the compression of dural and/or neural structures at the craniocervical junction or to the associated syringomyelia. Additionally, patients may exhibit symptoms and signs of associated disorders. CIM is a heterogeneous and multifactorial disorder including congenital and acquired forms; it can also be found as an isolated malformation or in association with many clinical conditions. We analyse the clinical features in a series of 65 children with CIM, focusing on the high frequency of associated clinical disorders. We emphasise the importance of a careful clinical and neurological assessment for a proper diagnosis and a correct management of these patients.
Neurological Sciences 12/2011; 32 Suppl 3:S295-8. · 1.32 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The Chiari malformation (CM) is a syndrome embodied in heterogeneous groups of malformations, spanning from the more benign and known, the CM1, to more complex syndromes such as the rare association with the tethered cord, as spinal lipomas, and the CM2, associated to open spina bifida. The clinical picture may be well expressed and detected at birth or even during intrauterine life, as for CM2, but in the other cases they may run a rather subtle clinical course. The diagnosis of these syndromes is driven by clinical examination and MRI, and it usually requires a multidisciplinary approach in order to plan the therapeutic strategies, such as surgery. Among the diagnostic investigations, the imaging techniques represent the most useful, for their capabilities to detect subclinical lesions, such as syringomyielia and lipoma; the urological investigation is useful to evaluate the urogenital dysfunctions. The neurophysiological investigations represent a non invasive diagnostic procedure to investigate the peripheral nerve, the spinal cord, the brainstem functionalities and more higher brain functions; the nerve conduction studies and the cranial reflexes, the brainstem (BAEP) and the somatosensory (SEPs) evoked potentials (EPs), alone or in combination, can be used for the diagnosis, follow-up and intraoperative monitoring. The most useful diagnostic tools in CM1 are likely represented by the brainstem auditory evoked potentials (BAEPs) and the blink-reflex (BR), while the usefulness of SEPs is still doubtful and debated; in CM2 and tethered cord the neurophysiological techniques can be combined in different ways in order to make a functional balance and to answer specific questions. BAEPs and BR can be useful to investigate the brain stem functionality and SEP to evaluate whether the ascending sensory pathway to the cortex can be hampered at some level; the visual EPs are particularly useful to evaluate the integrity of posterior visual pathway and visual cortex in the case of associated hydrocephalus. In the tethered cord, both nerve conduction study and somatosensory evoked potentials (SEPs) are useful to evaluate motor and sensory dysfunction of the lombosacral roots and nerves and spinal cord for their capability to detect subclinical impairment of conduction along the sensory and motor pathway. Finally, last but not the least, the neurophysiological techniques are remarkably useful during surgery; the intraoperative monitoring (IOM) by means of electromyography and direct nerve stimulation and recordings are able to detect early nerve damage, minimize nerve lesions and optimise the surgical techniques. In the operated children with incomplete removal of lipoma and/or persistent tethering, the recordings of SEP and BAEP are useful to demonstrate a conduction deterioration along the ascending sensory pathway due to increasing tethering of the spinal cord due to somatic growth.
Neurological Sciences 12/2011; 32 Suppl 3:S311-6. · 1.32 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The surgical results of this series of occult spina bifida seem better than the natural history registered in the long pre-operative period in terms of neurological deterioration. The major contribution to this result is attributed to neurophysiological monitoring that lowers the risks of permanent damage and increases the percentage of effective detethering. The present series of TCS, due to conus and filar lipoma, documents that CM1 is a really rare association occurring in less than 6% of the patients, despite the low position of conus. The detethering procedure did not influence the tonsillar position, thus excluding the correlation between the tethering and the tonsillar descent. The genetic alteration documented in a girl reinforces the hypothesis of a rare complex polymaformative picture deserving multiple procedures according to the prevailing clinical symptoms.
Neurological Sciences 09/2011; 32 Suppl 3:S353-6. · 1.32 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Chiari malformations 1, 2, 3 represent different degrees of herniation of posterior fossa content into the cervical canal (Chiari 1 and 2), or through an upper-cervical meningocele (Chiari 3), whereas Chiari 4 anomaly consists of hypoplasia of the cerebellum. Chiari 1 malformation (CM1) is the commonest anomaly; it is probably related to a mesodermal defect that create a congenitally small posterior fossa, subsequent overcrowding of its contents and herniation through the foramen magnum. The diagnosis of CM1 is based on the demonstration of the downward displacement and particular shape of the cerebellar tonsils into the upper cervical spinal canal associated with obliteration of the subarachnoid spaces at the level of the foramen magnum. MRI has a fundamental role in the correct identification of the anatomical aspects of this malformation and associated anomalies and represents the imaging modality of choice.
Neurological Sciences 07/2011; 32 Suppl 3:S283-6. · 1.32 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: In patients with Chiari malformation type 1 (CMI), epileptic seizures are occasionally reported both in symptomatic patients candidate to surgery and in patients without symptoms of tonsillar displacement in whom CM1 is often an incidental finding in the diagnostic work up for idiopathic epilepsies. In both groups of patients, the course of epilepsy is almost invariably favorable, with a few seizures easily controlled by treatment. In a subset of CM1 patients, epilepsy occurs in the context of neurodevelopmental disorders that also include mental retardation, autism and somatic dysmorphisms. Epileptic seizures must be accurately differentiated by potentially harmful paroxysmal events due to compression of the medulla, particularly by the cerebellar fits characterized by drop attacks, abnormal extensor posturing and apnea.
Neurological Sciences 07/2011; 32 Suppl 3:S303-6. · 1.32 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: To demonstrate the role of intraoperative multichannel electromyographic (EMG) monitoring to reduce postoperative deterioration and achieve full untethering of complex occult dysraphisms.
A retrospective analysis was performed on 66 patients who underwent operation for lumbosacral lipomas. Twenty recent cases were submitted to EMG monitoring and stimulation.
All patients presented symptoms at the time of surgery, and 74% exhibited progressive deterioration during the lengthy preoperative period. Postoperative surgery-related deterioration was observed in 6% of patients. This number was reduced to zero with the introduction of intraoperative EMG monitoring.
Intraoperative multichannel EMG monitoring can be carried out and requires only minimal changes to anesthetic procedures. With this method, it is possible to better identify the neural structures of complex malformations, reducing the risks of surgical damage and incomplete detethering.
Neurosurgery 05/2005; 56(2 Suppl):315-23; discussion 315-23. · 2.79 Impact Factor
