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A hazard of combining the infratentorial supracerebellar and the cerebellomedullary fissure approaches: cerebellar venous insufficiency.

The infratentorial supracerebellar approach is a popular technique for exposure of lesions of the superior vermis and pineal region. The cerebellomedullary fissure approach is enjoying increasing application as a technique for exposure of the fourth ventricle. Occasionally, a tumor that arises in the quadrigeminal plate or the superior vermis grows to fill the fourth ventricle, and for such a case a combination of the infratentorial supracerebellar approach and the cerebellomedullary fissure approach might be considered. We report a grave hazard of this combination. Two patients with tumors involving the superior vermis and filling the fourth ventricle were managed with a combined infratentorial supracerebellar/cerebellomedullary fissure approach. The first patient, who underwent a bilateral exposure, died on the sixth postoperative day due to massive hemorrhagic venous infarction of the cerebellum. The second patient, who was explored on one side only, suffered a protracted postoperative course characterized by suboccipital pain, torticollis, feeding difficulties and persisting hydrocephalus. Postoperative imaging showed swelling of the inferior vermis and ipsilateral hemisphere of the cerebellum with unilateral tonsillar herniation. Simultaneous compromise of the galenic and tentorial bridging veins and interruption of collateral pathways between these systems and the petrosal bridging veins, as in the combined infratentorial supracerebellar/cerebellomedullary fissure approach, may cause cerebellar venous insufficiency with venous congestion and possible venous infarction.

Adolescent↗

Low-dose botulinum toxin-a treatment of cervical dystonia - a double-blind, randomized pilot study.

The purpose of the study was to evaluate the clinical efficacy of low-dose botulinum toxin (BTX) treatment of cervical dystonia in a double-blind, randomized pilot study. Thirty-one patients with cervical dystonia and a minimum of 2 previous Dysport treatments received either a mean total target dose of 547 +/- 113 mouse units (MU) (group A, 500 MU Dysport/ml) or a 4-times-diluted preparation 130 +/- 32 MU (group B, 125 MU Dysport/ml). Assessment was made before and 4 weeks after the injection using Toronto Western Spasmodic Torticollis Rating Scales (TWSTRS). Additionally, a self-response scale evaluated the patients' subjective condition for 12 weeks after injection. TWST rating before and after injection revealed comparable clinical improvement in both groups. The severity of cervical dystonia at study entry had no effects on therapeutic effects of either preparation. Self-rating revealed comparable subjective improvement in both groups; however, 3 patients of group B received reinjections due to insufficient effects of the injection. Our findings suggest that low-dose treatment of cervical dystonia with Dysport may be clinically effective during maintenance therapy, at least for a limited period of time. Long-lasting effects of previous Dysport treatments at conventional doses and possibly improved diffusion of a highly diluted toxin preparation may have contributed to the effects of the low-dose regimen. Our data emphasize the need for further studies in order to clearly identify optimal toxin preparations for therapeutic uses of BTX-A in neurologic disorders.

Adult↗

Unusual neuroenteric cysts: diagnosis and management.

Six cases of unusual neuroenteric (NE) cysts, occurring in children aged 15 months to 18 years, are reported here. Three of the cysts were extramedullary, while the other three were intramedullary. Two of the extramedullary cysts were located at the lumbosacral region, an unusual site. Only one patient had the cyst located in a ventral relation to the cord in the cervicothoracic region. None of the patients had communication with the mediastinum or intraabdominal viscera. Stigmata of spinal dysraphism were seen in three patients. All the children had variable neurological involvement and it was difficult to differentiate NE cyst from other causes of spinal cord or cauda equina lesion, particularly in the absence of some dysraphic marker. One patient presented with minimal neurological deficits and painful torticollis; this patient was suspected to have an atlantoaxial dislocation. All patients were investigated with MRI, and the only diagnostic feature of an NE cyst was an intradural cyst with an anterior vertebral body anomaly in a single case. It was not possible to radiologically differentiate NE cyst from other intraspinal cysts in the rest of the patients. All patients were operated on by the posterior route; an attempt to excise the cyst nearly in toto was made. Evidence of neural tethering most probably due to cyst contents was seen in three patients. All patients improved after surgery. None showed recurrence of the cyst at follow-up of 3 months to 2 years.

Adolescent↗

Spinal cord stimulation revisited: psychological effects.

97 patients undergoing spinal cord stimulation (SCS) were studied for a median of 24 days after initiation of treatment. SCS was associated with significant improvement in memory, visuo-spatial integration, activation, cognitive efficiency, attention and overall psychological status. Significant emotional improvement in self-image, anxiety and depression was also confirmed following SCS. Diagnosis did not differentially affect psychological outcome except for relief of anxiety, which was less pronounced in the case of torticollis and multiple sclerosis. The role of spinal cord modulation of behavioral activation and physiological arousal was discussed.

Adolescent↗

Experience with 509 plate electrodes implanted epidurally from C1 to L1.

This article summarizes the experience gained with implantation of 509 plate electrodes performed by a single neurosurgeon. 350 patients were subjected to implantation of plate electrodes in the dorsal epidural space. 227 patients were implanted for chronic pain management (reflex sympathetic dystrophy, failed back syndrome/arachnoiditis, pain following spinal cord injury, nerve injury pain and other miscellaneous pain conditions), 105 patients for motor disorders (spasms/spasticity following spinal cord or head injury, cerebral palsy, multiple sclerosis, spasmodic torticollis and other miscellaneous conditions) and 18 patients for both. A total of 509 electrodes were implanted in the dorsal epidural space. The electrodes types were: 442 Medtronic Resume, 39 Medtronic Resume-TL and 25 Neuromed Lamitrode. 378 electrodes were implanted for chronic pain management, 106 for motor disorders and 25 in patients presenting with both pain and motor disorders. 192 electrodes were implanted in the cervical area and 317 in the thoracic area. 3.7% of the implanted electrodes became infected and had to be surgically removed. Electrode migration occurred in 1.1% of the patients and electrode breakage in 4 patients. 288 (70%) of the implanted electrodes are still being used. Technical factors relevant to the surgical implantation of plate electrodes at various levels in the spine are presented and discussed.

Adolescent↗

CT-guided ablative stereotaxis without ventriculography.

A new technique is described which permits all types of stereotactic surgery to be done without ventriculography, with CT guidance only. The present series consists of 42 patients who underwent thalamotomy, posteromedial hypothalamotomy, dentatotomy or anterior capsulotomy for movement disorders, chronic pain, spasmodic torticollis or psychiatric illness. Postoperative lesion control with repeated CT showed that the method was accurate. The clinical results were considered to be better than those after previous ventriculography-guided surgery. It is concluded that ventriculography is no longer needed for stereotactic neurosurgery.

Brain Diseases↗

Reversibility of chronic neurologic deficits. Some effects of electrical stimulation of the thalamus and internal capsule in man.

Stimulation of the thalamus and internal capsule with Medtronic deep brain stimulation electrodes produced improvement in pain, hemiparesis, dystonia, torticollis, tremor. speech impairment and epilepsy. Stimulation at voltages above or below clinically effective levels (e.g., 6 V, 0.3 ms, 74 Hz) resulted in a loss of clinical efficacy. Somatosensory evoked responses (short and long latency) and depth electrode recordings were helpful in localisation and 'biocalibration' of electrical stimulation.

Adult↗

Role of computerized transverse axial tomography on stereotactic surgery of the diencephalon. A preliminary report of 67 cases.

67 cases of various functional disorders of the diencephalon were examined by EMI scanner. The patients were composed of 38 cases of parkinsonism, 7 cases of thalamic syndrome, 6 cases of choreoathetoid movement, 2 cases of dystonia, 11 cases of involuntary movement of unknown etiology and 1 case of torticollis, tic, and ballismus, respectively. In parkinsonism, 79% showed diffuse cerebral atrophy, 5% had focal low density in the substantia nigra and the thalamus, whereas 16% remained normal. Pre- and postoperative assessment with CT scan was briefly discussed with reference to stereotactic surgery of the diencephalon.

Adult↗

Problems in childhood migraine.

There are differences between childhood and adult migraine which justify clinical research. First, inessential differences, such as prominence of systemic upset, indicate need for clinical drug trials. Second, some paroxysmal phenomena of childhood such as paroxysmal torticollis, vertigo, and hemiplegia may be migraine precursors, and need therapeutic trials. Third, some proposed equivalents of migraine in childhood need study and definition, followed by clinical trials.

Age Factors↗

Osteoblastoma of the odontoid process.

A case of an osteoblastoma located in the odontoid process in a 7 year-old boy who presented with torticollis is reported. CT scan and MRI disclosed the lesion, but diagnosis was established by a CT-guided needle biopsy. Surgery through a transoral approach allowed total excision of the tumor. This is, to our knowledge, the first case reported of an osteoblastoma of the odontoid process.

Bone Transplantation↗

Occipital plagiocephaly: deformation or lambdoid synostosis? I. Morphometric analysis and results of unilateral lambdoid craniectomy.

Between 1987 and 1992, 30 infants aged 1.4-13 months (mean 7.3 months) underwent unilateral lambdoid strip craniectomy at the Children's Hospital of Buffalo for occipital plagiocephaly. Males outnumbered females (22:8) and right-sided occipital flattening was significantly more common than left-sided flattening (25:5). The deformity was noticed at an average age of 3.2 months; 16% of the infants had an asymmetry at birth. Positional preferences (a distinct tendency to lie preferentially on the back, in most cases with the head turned to the ipsilateral side) were described in 79% of infants for whom this information was available, and torticollis was present in 10%. Pre- and post-operative CT scans were analyzed using several morphometric measurements. Asymmetries were measured between the flattened and contralateral sides, both posteriorly and anteriorly, using a translucent grid placed over the CT slice showing maximum asymmetry. The average maximum asymmetry between the flattened and contralateral sides was 24% posteriorly and 16% anteriorly. Significant improvements were seen postoperatively, with both anterior and posterior asymmetries improving by an average of one third (p < 0.05). However, when compared with CT scans from a control group of infants without synostosis, the operated group showed persistent and significant asymmetries postoperatively. The morphometric measurements described allow an objective and reproducible means of assessing the results of various treatments for this disorder. The improvements following unilateral lambdoid craniectomy are difficult to interpret in isolation; we suggest that future efforts be directed toward similarly assessing the results of both nonoperative treatments such as positional changes and molding helmets, and more aggressive surgical treatments that have been advocated for this disorder.

Craniofacial Abnormalities↗

Benign paroxysmal vertigo in childhood: a migraine equivalent.

The two main problems in defining and classifying the syndrome of benign paroxysmal vertigo (BPV) in childhood are the vestibular function pattern and the relationship between BPV and migraine. 13 children suffering from this syndrome were submitted to complete otoneurological examination, including caloric and rotational labyrinthine stimulation with ENG recording, and to headache provocation tests with nitroglycerin, histamine and fenfluramine. Vestibular responses were normal in all except 2 cases which presented signs of central vestibular impairment at the level of the vestibulocerebellar pathways. Headache provocation tests were positive in 9 out of 10 children, and in 4 cases they induced a typical vertiginous attack instead of headache. In addition, several children had a positive family history for migraine, headache was frequently associated with the crisis and other signs of a 'periodic syndrome' (motion sickness, cyclic vomiting, abdominal pain) were present, unrelated to vertiginous attacks. During the follow-up period, some children responded positively to migraine treatment. BPV, like paroxysmal torticollis in infancy and the signs of the periodic syndrome, can be considered a migraine equivalent or a migraine precursor and could be due to the same vascular and/or biochemical mechanisms responsible for the migraine. In children, for anatomical or developmental reasons, these mechanisms could selectively affect parts of the brain stem, including the vestibular nuclei and vestibulocerebellar pathways.

Child↗

Intraoperative suspension-recession technique for treatment of vertical strabismus in thyroid myopathy.

Ten patients underwent extraocular muscle surgery with an intraoperative suspension-recession technique for diplopia induced by vertical strabismus in thyroid myopathy. The average ratio of the reduction of vertical deviation to the amount of surgery was 1.8 degrees/mm (range, 1.0 degrees/mm-2.8 degrees/mm). Eight (80%) patients had single binocular vision restored in the primary position without prism or compensatory torticollis after surgery. One patient who showed evidence of abnormal thyroid function at surgery developed an overcorrection postoperatively.

Adult↗

Biofeedback in the treatment of blepharospasm: a case study.

Although biofeedback has been of value in treating spastic paresis and torticollis, its effectiveness in other movement disorders is less clear. Blepharospasm, which has no definitive treatment, often has a strong psychogenic component. The authors report the use of electromyographic and temperature biofeedback in a women with blepharospasm who had responded poorly to psychotherapy. Her improvement during biofeedback treatment further elucidated psychogenic factors contributing to the disorder. At 3-months follow-up it seemed that the patient's secondary gain from the blepharospasm diminished the maximum treatment response she could have had to biofeedback.

Biofeedback, Psychology↗

Klippel-Feil syndrome with conductive deafness and histological findings of removed stapes.

The Klippel-Feil syndrome is usually associated with sensorineural hearing impairment, but rarely is it associated with conductive or mixed deafness. A 22-year-old female presented with fusion of the cervical vertebrae, torticollis, scoliosis, pterygium colli, the Sprengel deformity with an omovertebral bone, concavity of the thorax and conductive hearing impairment of the right ear. Tympanotomy disclosed an atrophic long process of incus and a fixation of the stapes footplate, and stapedectomy was performed with immediate postoperative improvement of hearing. However, she developed a sudden hearing loss with dizziness soon after she had physical exercise on the 15th postoperative day, and revision surgery revealed a perilymph fistula of the oval window. Histological investigations of the removed stapes showed no specific osseous changes but hyperostosis of the posterior edge of the footplate. The literature is reviewed and the etiology of the conductive deafness and the perilymph fistula is discussed.

Adult↗

Behavioural effects of selective serotonin reuptake inhibitors following direct micro injection into the left red nucleus of the rat.

The behavioural effects of selective serotonin reuptake inhibitors (paroxetine, sertraline, citalopram, fluvoxamine, fluoxetine) and reference compounds (N,N'-di(o-tolyl)guanidine, haloperidol, 3-(3-hydroxyphenyl)-N-(l-propyl)piperidine and chlorpromazine) were studied for their ability to produce dystonia and torticollis following direct micro injection into the left red nucleus of the rat, an area of the brain containing a high density of sigma2 receptors but relatively devoid of biogenic amine receptors. Each animal was monitored for abnormalities in posture and movement for a period of 30 min and then sacrificed 40 min following drug administation. Only fluvoxamine (100 nmol) and fluoxetine (100 nmol) elicited acute dystonic behaviour (1-5 min). The onset of dystonia was accompanied by facial spasticity, vacuous chewing movements and grooming behaviour which reflected the extent of dystonia. The dystonic behaviour following the direct intrarubal injection of fluvoxamine and fluoxetine suggest the possible activation of sigma2 receptors while citalopram, sertraline and paroxetine were without effect. The results of this study support the role of sigma2 receptors in the regulation and control of movement and coordination and provides preliminary evidence to suggest the in vivo activity of sigma receptors by fluoxetine and fluvoxamine.

1-Naphthylamine↗

Crooked calf disease: a histological and histochemical examination of eight affected calves.

Macroscopic, histopathologic, and histochemical investigations were made on a group of eight neonatal Angus X Hereford calves, selected from an ongoing outbreak of crooked calf disease among calving heifers. Arthrogryposis of the forelimbs was seen to varying degrees in all eight animals, and torticollis was present in six calves. Histopathology, using hematoxylin and eosin stain, did not reveal any striking or consistent lesion in the affected animals; the majority of the tissues sampled were normal. Muscle samples were processed for adenosine triphosphatase (ATPase) and NADH-tetrazolium reductase (NADH-tr) histochemistry, and the data suggest that a primary myopathy is not responsible for the congenital anomalies in the affected calves.

Animals↗

Cervico-pectoral ectopia cordis in two Holstein calves.

Two Holstein calves affected with cervico-pectoral ectopia cordis, a male (No. 1) delivered stillborn and a female (No. 2) died 1 hour after birth, were examined macroscopically and radiographically to assess the severity and elucidate the teratogenesis of the anomaly. The heart of one calf was covered by the intact pericardium and skin and displaced to the caudalmost portion of the ventrocervical region, just cranial to an enlarged thoracic inlet. The cranial vena cava and the vena azygos were duplicated. The sternum was bilaterally divided into two parts fused only at the xyphoid process and was semicircular. The heart of the other calf, covered solely by the pericardium, was exposed ventrally from an oval opening in the body wall just cranial to a defective Y-shaped sternum. In both calves, the arterial branching pattern from the aortic arch was intermediate between the patterns of the dog and pig, and in each case a single pulmonary vein emptied into the left atrium. Pronounced or slight torticollis, cleft palate, and abnormalities in the urogenital system were also found. From an embryological perspective, cervical and pectoral ectopia cordis have a common pathogenesis; the cervical type occurs at a slightly earlier fetal stage than the pectoral type.

Animals↗