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PubMed1976

[Differential diagnosis of dizziness (author's transl)].

The common denominator of the different meanings of dizziness is a disturbance of spatio-postural orientation, which indicates an imminent danger of fall. Höhenschwindel (fear of hights) with grasping and holding in the primate is instinctive behavior caused by a visual cliff or sudden dizziness. Because of the many possible meanings of dizziness, the case history is important for differential diagnosis. Vertiginous and non-vertiginous dizziness, black out and unsteady gait without vertigo should be distinguished. In addition, the duration and time course of the attack, releasing mechanisms and accompanying symptoms of dizziness should be explored. In recurrent dizziness without hearing loss, vestibular, vascular, cardial and epileptic disorders should be considered as well as intoxication, cerebral tumor, cerebellar hemangioblastoma, multiple sclerosis, neurosis and psychosis. Epileptic dizziness occurs not only in the prodromal stage of grand mal attacks and in temporal lobe epilepsy, but also in petit mal absences of short duration (less than 5 s) in which the blurring of consciousness is not apparent; absences of short duration are easily overlooked in childhood. Besides the objective history obtained from the patient's relatives, EEG-recording when falling asleep in the morning after one night of sleep deprivation are the best means for the diagnosis of epilepsia. Spontaneous nystagmus after complete exclusion of visual fixation is a physiological phenomenon. For differential diagnosis between physiological and pathological spontaneous nystagmus, Frenzel's spectacles in the dark room are indispensable. The distinction of spontaneous nystagmus in the narrower sense, gaze nystagmus and fixation nystagmus is discussed. The diagnostic importance of the direction of nystagmus is mentioned. Jerking nystagmus may be congenital. Pendular nystagmus may be acquired. Therefore, additional criteria for the differential diagnosis between congenital and acquired nystagmus are necessary.

Alcoholism↗
PubMed1986

Cranio-cervical growth collision: another explanation of the Arnold-Chiari malformation and of basilar impression.

Analysis of neuro-cranio-spinal development suggests a cranio-cervical growth conflict as the cause of the Arnold-Chiari malformation and of basilar impression. The ascending course and elongation of the upper cervical nerves associated with the Arnold-Chiari malformation reflects the abnormal, caudo-cranially proceeding growth of the cervical spine. This is the opposite of the normal cranio-caudal direction of growth (which includes the brain) with downward slanting of the cervical nerve roots. The cervical growth reversal is a compensatory event related to the impairment of distal spinal growth at the level of the coexistent myelomeningocele. With the reversal of the cerical growth, the initial descent (uncoiling) of the primordial brain curvatures is compromised owing to the growth-collision with the ascending cervical spine. Their subsequent growth proceeds into the upper cervical spinal canal. The contents of the posterior cranial fossa are actively "sucked up", "devoured" by the latter. In contrast to the adaptively enhanced growth of the early cranio-cervical nervous structures in the Arnold-Chiari malformation, as an answer to the growth-shifts of the encasing skeleton, basilar impression is a postembryonic adaptation of the cervico-cranial skeleton to the inadequate growth of the nervous structures after the latter have lost their growth adaptability. Arnold-Chiari malformation and basilar impression are just two representatives of "osteo-neural growth pathology" encompassing some "dysplastic" disorders of the axial as well as of the limb skeleton such as platyspondyly, scoliosis, Scheuermann's kyphosis, achondroplasia-like conditions, congenital dysplasia of the hip etc.(ABSTRACT TRUNCATED AT 250 WORDS)

Arnold-Chiari Malformation↗
PubMed1976

Chronic tonsillar herniation: an attempt at classifying chronic hernitations at the foramen magnum.

A system is presented for the classification of chronic herniations of the cerebellar tonsils in the absence of space-occupying intracranial lesions, based on a survey of the literature and 13 own cases. The Arnold-Chiari malformation in adults typically involves herniation of the cerebellar tonsils instead of herniation of the vermis as is typical when it occurs in infancy. Identification of chronic tonsillar herniation with the Arnold-Chiari malformation in adults was thought to require at least one other sign of the Arnold-Chiari complex, e.g. a medullary deformity. Cases for which chronic herniation and sclerosis of the cerebellar tonsils present as the only nervous lesion are classified as "chronic tonsillar herniation". Such cases may manifest with neurological symptoms during adult life, or they may be found incidentally at autopsy. Attention is drawn to the occurrence of chronic tonsillar herniation in 7 infants and children where it apparently represents a cause of sudden unexpected death. The overlap between chronic tonsillar herniation and the Arnold-Chiari malformation of adults is discussed in regard to the frequence of associated osseous anomalies, hydrocephalus and syringomyelia.

Adolescent↗
PubMed1993

Unusual mechanism of lethal cervical spinal cord injury in a case of atlanto-axial diastasis.

A case of traumatic cervical spinal cord injury due to congenital craniocervical abnormalities is described. The autopsy revealed evidence of hyperextension of the neck due to frontal impact, congenital occipitalization of the atlas accompanied by basilar invagination of the odontoid process and atlanto-axial diastasis. The subsequent narrowing of the spinal canal led to a predisposition for this unusual mechanism of spinal cord injury.

Accidents, Traffic↗
PubMed1992

Craniocervical junction malformation treated by transoral approach. A survey of 25 cases with emphasis on postoperative instability and outcome.

An experience with 25 consecutive cases of craniocervical junction (CCJ) malformations operated upon via the transoral route is reported. Twenty-two patients also underwent posterior occipito-cervical stabilization with alloplastic material and in only one patient was transoral odontoidectomy and fusion with bone autograph performed. Indication for the transoral route consisted of an irreducible ventral compression of the cervicobulbar junction by the abnormal bone complex. Two patients died during the early postoperative period and the remaining 23 survivors were followed for an average of 3.5 years: 17 of these showed marked improvement and 5 a stabilization of the neurological disturbances. A further patient, who refused posterior stabilization, eventually died because of progressive cranial settling. Long-term results have shown this approach to be decisive in the surgical management of well-selected CCJ anomalies.

Adult↗
PubMed1988

Exophthalmos and basilar impression. A contribution to differential diagnosis of endocrine orbitopathy.

We report on a male patient with exophthalmos of unclear etiology, basilar impression, syringohydromyelia and type II Arnold-Chiari malformation. Two diseases involving the orbital region were to be considered in differential diagnostic terms: endocrine orbitopathy and osseous orbit dysplasia. The typical physical appearance associated with basilar impression as well as suppurative keratitis in Lagophthalmos was striking. Tetraspasticity with pareses, bulbar symptoms, proximally pronounced muscular atrophy as well as a left hemihypesthesia was shown neurologically. Although the orbit CT was normal, sonography revealed thickened ocular muscles. There was euthyroidism in diffuse goiter with negative thyroid autoantibody findings. Because of lack of definitive detection of muscular swelling, tumor, or vascular anomaly in the various images, orbital dysplasia in the context of a malformation syndrome affecting several organs is suggested as the cause of the exophthalmos.

Arnold-Chiari Malformation↗
PubMed1983

Craniocervical malformations.

This short survey summarises the important craniocervical dysplasias, the malformations accompanying primary neuroectodermal and secondary lesions of the neural structures and their coverings, and complex malformations. The interpretation, classification and the identification of the static and/or dynamic biomechanical pathogenesis is a prerequisite for the correct diagnosis and for assessing the indications for and the choice of any operation to be adopted.

Axis, Cervical Vertebra↗
PubMed1983

Computer-tomographic investigations in malformations of the occipito-cervical junction.

The diagnosis of occipito-cervical dysplasias has been improved markedly by computer-tomography. Two millimetre transverse cuts with sagittal and coronal reconstructions are necessary. Based on nine cases with nearly all types of malformations it is shown that even complex osseous (mesodermal) malformations and dysplasias as well as isolated or combined neuroectodermal dysplasic or secondary compressive lesions of the medulla oblongata and cord can be detected. Transverse and AP diameters of the foramen magnum and spinal canal and the classical lines and angles can be measured. This time-consuming procedure will give more information than the classical radiological methods and avoid myelographic and cisternal examinations. Its disadvantages are the impossibility of dynamic functional studies and the length of the procedure.

Adolescent↗
PubMed1983

Craniocervical abnormalities. Modern diagnosis and a comprehensive surgical approach.

A retrospective study of 41 patients operated on between 1975 and 1983 is presented including guidelines for surgical management with some alternative technique both in anterior and posterior approaches and modern diagnostic procedures. A proposed base-line inclined--10 degrees from Reid's base-line proved to be adequate to obtain the true shape and size of the foramen magnum on CT-scan. The normal range of the clivo -axial angle in adults is reported and its clinical significance is stressed.

Adolescent↗
PubMed1983

Transoral operations for craniospinal malformations.

The transoral approach to the lower third of the clivus and to the ventral aspect of the upper cervical spine is used in craniospinal malformations with or without dislocation as well as in basilar aneurysms, ventrally situated cranio-spinal tumours, fractures of the odontoid process, and in rheumatoid arthritis compressing the spinal cord. In consideration of the literature and ten personal cases the indications and techniques of the transoral approach in craniospinal malformations are discussed. According to our own experiences and those of other authors it is possible to expose the lower clivus and the cervical spine down to C2 by a midline incision of the pharyngeal wall using a mouth retractor and oral intubation. Splitting of the soft palate or resection of the hard palate are not necessary, a tracheotomy should be performed only in exceptional cases. In congenital craniospinal malformations without dislocation or instability causing a ventral compression of the spinal cord, for instance by the odontoid process, the transoral decompression is preferable to dorsal decompressing operations. In cases of pure instability without any space-occupying lesion the transoral and posterior approach are possible in order to perform a fusion. The last one seems more advantageous in these cases. In craniospinal malformations with dislocation causing a ventral and dorsal narrowing of the spinal canal, apart from the decompression a stabilization has to be achieved. In these usually complex malformations individual treatment is necessary. According to the rare cases in the literature and to our own experience a primary anterior decompression, followed by a most careful posterior stabilization seems to produce the most favourable results.

Adult↗
PubMed1979

The subforaminal stenosis headache.

The clinical picture of the so-called subforaminal stenosis headache (Gutmann) and its surgical treatment (Roesner) have been presented. We have analyzed our findings in 119 patients and 55 operations. The clinical picture is characterized by increasing pain in the upper cervical and occipital region which in later stages may be accompanied also by other symptoms like disturbances of concentration, memory, libido, and potency, as well in some cases by symptoms similar to those of a secondary chronic myelopathy. The syndrome is caused by a subforaminal stenosis of the dural sac which regularly is compressed by the posterior atlas arch in connection with either morphological variations of the base of the skull or by static-functional deviations of the cranio-cervical region. The compression of the dural sac of the cranio-cervical region results in an impairment of its air chamber function, and additionally may disturb the connections between the intracranial and intraspinal venous plexus. By both factors the physiologically important smoothing function of the CSF space of the cranio-cervical region concerning intracranial pressure changes is disturbed. Treatment of choice is a laminectomy of the dorsal arch of the atlas and an osteoclastic dilatation of the foramen magnum but without opening of the dura. The results of this procedure are excellent.

Adult↗
PubMed1994

Congenital malformations of the craniovertebral junction: classification and surgical treatment.

The clinical, radiological and surgical findings of 56 patients with congenital malformations of the craniovertebral junction who underwent surgical intervention in the Department of Neurosurgery, Hacettepe University Medical School were reviewed. The anomalies were classified according to their embryonic origin. Forty-two cases of the series were major abnormalities formed by the combination of more than one germ layer.

Adolescent↗