[Implementation of a competency-based graduate medical education program in a neurology department]. / Einführung eines kompetenzbasierten Facharztweiterbildungsprogramms in einer neurologischen Abteilung.

BACKGROUND: Competency-based programs have long been the basis of graduate medical education in British and North America. Such formally defined program structures are lacking in German graduate medical education. METHODS: The methods and assessment tools used in a curriculum-based program of graduate education in medical specialties are presented. The feasibility of the program was tested in day-to-day use in a pilot study conducted within a neurology department. Experience of it during a 1-year period after implementation has also been collected. RESULTS: It was found that implementation of the program is feasible providing that both the trainers and the students are well motivated, that adequate resources are provided, and that a formal training system is provided for the trainers. This is confirmed by the 1-year experience of the program in routine clinical practice. CONCLUSIONS: The competency-based program presented, which is compatible with many quality management systems, offers management and assessment tools that will make for more satisfactory postgraduate medical education.

The value of different neuro-imaging methods in the diagnosis of a congenital, spinal, epidural meningeal cyst.

Congenital, spinal, epidural cysts are rare causes of transverse or radicular spinal lesions. Usually these cysts are located in the thoracal region, are oblong-shaped, and extend over several segments dorsally to the cord. Frequently, they cause no symptoms for years. They are fairly accessible to diagnostics by modern neuro-imaging methods. We report on a 15 year old girl with paraparesis, which showed a slow progression over two years, short-term remissions, and finally impairment of bladder function. Myelogram, computer-assisted tomography and nuclear magnetic resonance tomography showed a large space-occupying mass from D8 to L1 epidural and dorsal of the myelon. Operation disclosed an epidural cyst from D10 to L2. Histologically, the removed material was classified as a meningeal cyst. Post-operatively the girl's condition improved under intensive physio-therapy. For the diagnosis of this spinal dysrhapic disorder CT scanning after intrathecal application of contrast medium and magnetic resonance imaging were most valuable.

[Therapy of orbital and retroorbital space occupying processes with optic nerve compression]. / Therapie orbitaler und retroorbitaler raumfordernder Prozesse mit Sehnervkompression.

Space occupying lesions involving the optic nerve require individual management. Graves' disease is first treated with cortisone: failures of treatment require radiation and operative decompression. Good results have been achieved with cytostatic drugs and, in acute progressive loss of vision, with plasmapheresis. Idiopathic pseudo-tumor orbitae disappears with cortisone, failures of treatment indicate malignant development of a lymphoma, which requires management with cytostatic drugs or radiation. Granulomatous infiltration of the optic nerve in sarcoidosis should be treated first of all with steroids before proceeding to operative treatment. Primary optic nerve tumors such as gliomas and meningeomas should be removed operatively if they are located in front of the chiasm and continue to grow. If the chiasm is involved, radiation is preferable. Doubtful cases of clinically almost indistinguishable pinealomas which are very radiosensitive require primary radiation therapy. Sinus cavernosus fistulas should be treated by selective angiographic occlusion of the fistula. If this is impossible, the internal carotid artery may be occluded by introducing a ballon catheter. Loss of vision after blunt head trauma should be treated with megadoses of steroids, operative treatment is only promising in cases with progressive loss of vision.

[Nuclear magnetic resonance tomography and computed tomography of the brain for detection of Wilson's disease]. / Kernspintomographie und Computertomographie des Gehirns zum Nachweis des Morbus Wilson.

The authors report on two woman patients with confirmed Wilson's disease (hepatolenticular degeneration) who had neurological deficits and showed typical changes evident from laboratory data. Both computed tomography and magnetic resonance tomography revealed degenerative changes in the basal ganglia, especially of the lenticular nucleus, MR showing these defects more clearly than CT. There was a noticeable symmetrical enhancement of signals in the lenticular nucleus which was particularly evident on the image basing on the T2 (spin-spin relaxation time constant) values. MR could be superior to CT with regard to showing up pathological changes in the basal ganglia. The future indication of MR could be the establishment of an exact correlation between clinical signs and symptoms on the one hand, and morphological findings on the other. Over and above this, it should be explored to what extent MR can already detect degenerative changes in the brain in primarily hepatic types of the disease even without prior neurological examination.

[Opsoclonus and myoclonus syndrome. Clinical and electronystagmography findings with course studies]. / Opsoklonus- und Myoklonie-Syndrom. Klinische und elektronystagmographische Befunde mit Verlaufsstudien.

This review is based on the clinical experience with 7 patients and a total of 171 additional cases described in literature. Clinical features are presented of this "benign" cerebellar encephalopathy, which occurs preferably in association with viral infections, neuroblastomas or rarely carcinomas. Numerous descriptions of single cases stress a heterogeneous variety of other diseases which possibly may cause this syndrome. Special emphasis is put on the electronystagmographic documentation of eye movements with follow up of the spontaneous recovery over several years. The eye movement characteristics of opsoclonus allow for a clear differentiation from other forms of acquired ocular oscillations, such as ocular flutter, ocular dysmetria, square wave jerks, macro-saccadicoscillations, lightning eye movements, pendular nystagmus, obliquus superior myokymia and voluntary nystagmus. Differential diagnosis of the underlying diseases is particularly dependent on the age of onset. Histopathological findings as well as the hitherto suggested immunologic pathogenesis, therapeutic regimen and the prognosis are discussed critically on the basis of the literature.

Modulation of vestibulo-ocular reflex (VOR) by active stabilization of head to trunk.

Horizontal vestibulo-ocular reflex (VOR) with covered eyes was tested in 15 healthy subjects sitting on a rotation chair (40 degrees, 0.05, 0.1, 0.2, 0.33 Hz) under two conditions: (a) head fixed to chair by a neck support; (b) with instruction to turn the head together with the passively moved trunk. In situation (b), saccadic amplitudes/sec, nystagmus frequency, gain of nystagmus slow phase velocity and amplitudes of maximum eye shift were enhanced significantly. The phase of maximum eye shift changed from 180 degrees up to 270 degrees with respect to stimulus position. The distribution of nystagmus saccades over the stimulus period showed enhanced peak values and a slight phase shift.

[Treatment of typical trigeminus neuralgias. Overview of the current state of drug and surgical therapy]. / Die Behandlung der typischen Trigeminusneuralgie. Uberblick über den aktuellen Stand der medikamentösen und operativen Therapie.

Carbamazepine continues to be the most useful drug in the treatment of trigeminal neuralgia. Diphenylhydantoin may be given in addition to or instead of Carbamazepine. Refractory cases may benefit from combination with Baclofen or Chlorphenesin. In cases of persistent pain the concomitant use of tricyclic antidepressant drugs is recommended. If pain continues in spite of multiple medical therapies or if serious side effects develop, then surgical procedures such as percutaneous controlled thermocoagulation or microvascular decompression are indicated. Percutaneous thermocoagulation is associated with the lowest mortality and morbidity rate and can easily be repeated. Microvascular decompression should especially be offered to young patients, who want to avoid any sensory disturbance of the face, and recommended for other patients for whom all other forms of therapy including percutaneous thermocoagulation have failed.

Motion perception of head or trunk modulates cervico-ocular reflex (COR).

In 15 sitting volunteers with eyes covered the vestibulo- and cervico-ocular reflexes were tested with sinusoidal movements around the vertical axis of the body, at frequencies of 0.05, 0.1 and 0.2 s-1 and a total amplitude of 40 degrees. When the trunk was moved against the fixed head, the subjects described mainly an illusionary head movement at 0.05 s-1, while, at 0.2 s-1 only the trunk movement was perceived. Subjects asked to imagine head motion during COR showed increased eye shifts and total saccadic amplitudes. With concentration on trunk movement perception these values decreased.

Cervico-ocular responses (COR) during slow sinusoidal head movements in subjects with bilateral labyrinthine lesions.

Eye movements in five patients with chronic bilateral labyrinthine loss were tested with sinusoidal movements during cervico-ocular stimulation and active head movements (0.05, 0.1 and 0.2s-1; 20, 40, and 60 degrees) and were compared with healthy subjects. Consideration was given to saccadic activity and slow phase velocity of nystagmus and overall gaze shift. The cervico-ocular response was not altered in the patients. During active pendular head movements the saccadic activity and slow phase velocity of nystagmus were more reduced than the eye shifts. The phase relation of eye shifts was not changed. In our patients neck to eye responses did not compensate for the abolished vestibulo-ocular reflex.

The effect of cervical and vestibular reflexes on eye movements in Huntington's chorea.

In 8 patients with manifest Huntington's Chorea vestibulo-ocular (VOR) and cervico-ocular (COR) reflexes were compared with eye movements during active head turnings. Seated patients were stimulated with their eyes closed by sinusoidal swings around the vertical axis at frequencies of 0.05, 0.1 and 0.2s-1 with amplitudes of 20, 40 and 60 degrees. 1) With all stimuli and in all patients a weak nystagmus was elicited in the direction of head movements, superimposed on larger slow eye deviations. 2) The averaged total saccadic amplitudes were smaller than in normals, increased with stimulus amplitudes and were smallest for COR, followed by VOR and active head movements. 3) The gain (peak velocity of slow phase of nystagmus to peak stimulus velocity) was only slightly below norm values and decreased with increasing stimulus frequency and amplitude. 4) The peak amplitudes of average slow eye deviations increased with stimulus amplitudes. In VOR they were comparable to norm values but were below them during COR and active head movements. 5) In normal subjects these slow eye deviations were compensatory to head movements in VOR but anticompensatory in COR and during active head movements. In choreic patients during COR and more often during active head movements these slow eye movements were compensatory for the head turning.

Vestibulo-ocular reflex (VOR), cervico-ocular reflex (COR) and its interaction in active head movements.

In normal adults the vestibulo-ocular reflex (VOR) and the cervico-ocular reflex (COR) were investigated during passive and active head or body movements, respectively. Sinusoidal rotations around the vertical axis of the body at frequencies of 0.05, 0.1, and 0.2s-1 and total amplitudes of 20 degrees, 40 degrees, 60 degrees, or 80 degrees were employed. The average eye deviations (Schlagfeld) during VOR were directed opposite to the direction of the head turning. During COR, however, slow eye deviations of higher amplitude were anticompensatory relative to the fixed head. During active head turnings the average eye deviations showed the same anticompensatory direction as in COR, but were still larger. The increased with stimulus amplitudes up to 60 degrees. At least a weak cervical nystagmus was elicited in all subjects, with its fast phases beating in the direction of the relative head movement. Its gain reached marked values up to 0.5, but only for peak stimulus velocities below 25%. The nystagmus gain during active head turnings was only slightly higher than during VOR. With higher stimulus velocities, large anticompensatory saccades appeared just before the change of stimulus direction; these are typical for active head movements, but were also found during COR.