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Visual loss caused by pseudotumor cerebri in an infant on peritoneal dialysis.

Infants with chronic renal insufficiency have multiple risk factors for developing pseudotumor cerebri (PTC) and are at particular risk for being diagnosed with PTC late, because of their inability to express symptoms. We describe a 13-month-old infant dependent on peritoneal dialysis, without evidence of central nervous system infection or inflammation, who developed acute vision loss secondary to PTC. Signs of PTC in infants include torticollis, inattentiveness, inability to track, facial paresis, or new-onset strabismus. Physicians responsible for the care of children with renal failure should be aware of the potential for PTC, as the diagnosis should be made as early as possible to prevent permanent visual loss.

Brain↗

[Botulinum toxin in specific pain therapy].

Botulinum toxin has been used for therapeutic purposes in medicine for more than 20 years. Its effective use now covers more than 50 conditions in a wide variety of areas. Its medicinal use was initially based on its blockade of neuromuscular and neurosecretory transfers. Its use for conditions in the field of specific pain therapy is currently authorized in Germany for spastic torticollis, blepharospasm, hemifacial spasm, spastic equine gait in cases of idiopathic cerebral paresis, and spasticity of the arm following stroke. New publications suggest that it can usefully be employed for numerous other painful conditions. The modes of action known today are not confined to the blockade of cholinergic innervation.Indeed, there is also evidence that therapeutic effects are mediated through a normalization of muscle spindle activity, retrograde intake into the CNS with modulation of the central neuropeptide function, inhibition of sterile neurogenic inflammation, and normalization of endplate dysfunction. In view of the methodological peculiarities of studies in the field of pain therapy, such as injection techniques, injection sites, blind study techniques, dosage etc., the scientific evidence for its use in a wide variety of pain syndromes is still patchy in many areas. For this reason the use of botulinum toxin for these syndromes is only justified after full use has been made of standard therapeutic methods and evaluation in specialized centers. The possibility of considering botulinum toxin in specific pain therapy contexts is a new option for patients and doctors.However, its use calls for detailed knowledge of functional neuroanatomy and extensive practical experience and expertise.

Analgesics↗

Excision of an osteoid osteoma from the body of the axis through an anterior approach.

Osteoid osteoma is seen less commonly in the vertebral body than in the posterior elements of the spine. To the authors' knowledge, this is only the second reported case of an osteoid osteoma affecting the body of the axis. The patient described in this report presented with the classic symptomatology of pain and torticollis. Radiological imaging confirmed the diagnosis. A superior extension of the anterior Smith-Robinson approach, through the "window of access" described by Fasel, was used to excise this lesion. Following surgical excision, there was complete resolution of the presenting symptomatology and functionally the range of motion of the cervical spine returned to normal. We avoided instrumentation and C1-C2 fusion so that rotation of the cervical spine could be maintained. There were no signs of recurrence of the tumour within the 4-year follow-up period. It presented in a classic way but, despite this, it took 2 years from the onset of these symptoms to reach a definitive diagnosis. This osteoid osteoma was successfully excised through a superior extension of the anterior Smith-Robinson approach.

Adolescent↗

Dystonia.

Therapy for most people with dystonia is symptomatic, directed at lessening the intensity of the dystonic contractions. For a small minority of patients (eg, those with dopa-responsive dystonia, Wilson's disease, or psychogenic dystonia), specific therapy directed at one of the many causes of dystonia is available. Before initiating treatment, clinicians need to decide if a patient has a form of dystonia amenable to such therapy. The most sensitive and least costly method to diagnose DRD is a therapeutic trial of levodopa. It is, therefore, recommended to treat all those with dystonia beginning in childhood or adolescence with low-dose levodopa. For patients with generalized or segmental signs who do not respond to levodopa, other oral medications, including anticholinergics, baclofen, and benzodiazepines, may provide mild to moderate relief; these medications are often given in combinations. For those with focal dystonia, most having adult-onset disease, botulinum toxin A injections often effectively control contractions. The injections produce transient weakness and need to be repeated, generally every 3 to 5 months. There is growing renewed interest in surgical treatment. Peripheral denervating procedures may be helpful for patients with torticollis who do not obtain adequate benefit with botulinum toxin A. The central procedures of pallidotomy and pallidal stimulation are under study; their place in the treatment of the many dystonia subtypes (eg, limb vs axial, generalized vs focal, primary vs secondary) still needs to be established. There are very few studies evaluating physical and psychological therapies or the impact of diet or lifestyle in dystonia. Most clinicians consider physical therapy, including massage, a potential adjunct to medical therapy, and psychological support and stress reduction may help individuals cope with this chronic and frequently disabling condition.

Journal Article↗

Effect of isolated inferior oblique muscle myectomy in the management of superior oblique muscle palsy.

Thirty-three patients with superior oblique palsy were treated with an inferior oblique myectomy temporal to the inferior rectus muscle. The mean effect of surgery was 11.91 +/- 1.38 in all positions of gaze and for all age groups. The operative result in both children and adults was the same. The mean effect of surgery correlated with the size of the preoperative hyperdeviation in the fields of action of both inferior and superior oblique muscles. Inferior oblique muscle myectomy was followed by return of normal function to the superior oblique muscle in 63% of cases. Ocular torticollis was eliminated in 90% of the patients. Persistent overaction of inferior oblique muscle was encountered in three patients. Postoperative underaction of the myectomized muscle was not observed in any of our patients. A myectomy is recommended as a primary procedure in patients with superior oblique muscle palsy when the direct antagonist is overacting and the hyperdeviation is 10 to 15 prism diopters or more in primary position or the fields of action of the paretic superior oblique muscle or the overacting inferior oblique muscle.

Adolescent↗

Pediatric rehabilitation. 6. Musculoskeletal and soft tissue disorders.

This self-directed learning module highlights advances in evaluation and treatment of congenital and acquired musculoskeletal disorders in the child. It is a section of the chapter on pediatric rehabilitation for the Self-Directed Medical Knowledge Program Study Guide for practitioners and trainees in physical medicine and rehabilitation. This section contains information on alterations of limb structure and gait, scoliosis, torticollis, sports injuries and overuse syndromes, and unexplained pain in children.

Arm Injuries↗

Cervical spondylotic myeloradiculopathy in dystonia.

Previous reports have attributed the development of premature cervical spondylosis to movement disorders such as torticollis and athetosis. This case report describes the clinical, electromyographic, and radiographic findings in a 34-year-old man who developed a myelopathy and cervical radiculopathy superimposed on a chronic dystonia of his neck and left arm. Cervical myeloradiculopathy should be suspected in any patient with a chronic movement disorder of the hand, neck, or arms, who presents with neurologic deterioration. Early diagnosis will lead to treatment that may improve symptoms. Anterior cervical bony fusion appears to be the preferred surgical treatment in these patients.

Cervical Vertebrae↗

Shunting in chronic post-traumatic hydrocephalus: demonstration of neurophysiologic improvement.

The neuronal metabolic state that forms the cellular basis for cognitive functioning and motor behavior is dependent on cerebral perfusion. As intracerebral pressure increases, cerebral perfusion is compromised. The management of acute hydrocephalus after head trauma has been extensively described. In this article, we present evidence of clinical, cerebral perfusion, and neurophysiologic improvement in a 19-year-old patient with chronic post-traumatic hydrocephalus. Lethargy, gaze palsy, torticollis, and triplegia were noted 2 years postinjury in a neurologically stable patient. Computed axial tomography (CAT) scan confirmed marked enlargement of the third and fourth ventricles with a prominent subgaleal fluid collection. Intraoperative cerebral spinal fluid pressure was 26cm H2O despite hyperventilation. A ventriculo-peritoneal shunt was placed. Postoperatively, marked improvement in clinical exam, cerebral perfusion (SPECT scan), and evoked potentials correlated with the reemergence of the cerebral mantle on CAT scan and magnetic resonance imaging (MRI). Chronic obstructive hydrocephalus should be suspected and aggressively pursued in chronic head injury.

Activities of Daily Living↗

Motor organization of the spinal accessory nerve in the monkey.

The segmental and topographical organization of motoneurons of the spinal accessory nerve was studied in monkeys with horseradish peroxidase (HRP). The sternomastoid muscle is innervated primarily from the C1-C2 level of the cord and the motor column is medial in the ventral horn. The trapezius muscle, however, is innervated primarily from the C2-C3-C4 levels of the cord and the motor column lies in the ventrolateral region of the ventral horn. The data are discussed with regard to treatment of torticollis.

Animals↗

Destruction of the nucleus raphe obscurus and potentiation of serotonin-mediated behaviors following administration of the neurotoxin 3-acetylpyridine.

Systemic administration of the neurotoxin 3-acetylpyridine (3-AP) to rats produced spontaneous episodes of spasmodic movement involving the trunk and limbs including torticollis, contortions of the trunk and rigid extension of the limbs. Because the neurotransmitter serotonin (5-HT) has been implicated in various human involuntary movement disorders, the functional and anatomical integrity of the 5-HT system in rats treated with 3-AP were examined. 5-HT-containing neurons in the brain stem were studied using immunohistochemical labeling with antiserum to 5-HT. Cells in the nucleus raphe obscurus were found to be altered following 3-AP treatment as shown by a decrease in 5-HT immunoreactivity as compared to control rats. No changes in 5-HT immunoreactivity were observed in any other region containing 5-HT cell bodies. Behaviorally, rats treated with 3-AP were 2.5-fold more sensitive to the ability of the 5-HT1A agonist 8-OH-2-(di-n-propylamino)tetralin (8-OH-DPAT; 0.33-3.3 mg/kg) to produce the 5-HT syndrome. Similarly, 3-AP-treated rats were 2-fold more sensitive to the selective 5-HT2 agonist 1-(2,5-dimethoxy-4-bromophenyl)-2-aminopropane (DOB; 0-1.0 mg/kg) at producing the head shake response. Although these behaviors associated with brain stem 5-HT receptors were potentiated by 3-AP, the hypothermic effect of 8-OH-DPAT which involves ascending mesencephalic 5-HT neurons was unchanged following 3-AP treatment. Treatment with 3-AP did not produce significant alterations of 5-HT or 5-hydroxyindoleacetic acid (5-HIAA) content in any brain region studied. Quantitative autoradiographic analysis of the density of 5-HT1A receptors labeled with [3H]8-OH-DPAT revealed that these sites were unchanged in regions of the brain (frontal cortex, hippocampus and brain stem) and in the spinal cord. Similarly, few changes in the density of 5-HT2 receptors measured with [3H]ketanserin were observed in various brain regions. These results suggest that neurons from the nucleus raphe obscurus are involved in the elicitation of 5-HT-mediated behavioral responses by 5-HT1A and 5-HT2 receptor agonists that are though to be mediated through brain stem and spinal cord mechanisms. In addition, because of the close neuroanatomical relationship of the nucleus raphe obscurus with various brain regions known to be involved in motor control, the destruction of this region by 3-AP may contribute to the spasmodic motor behaviors observed following 3-AP treatment.

8-Hydroxy-2-(di-n-propylamino)tetralin↗

La maladie de Grisel: a rare occurrence of "spontaneous" atlanto-axial subluxation after pharyngoplasty.

A review of the literature, with a short discussion of the possible aetiology, diagnosis and treatment of "la maladie de Grisel" is presented with a case report of atlanto-axial subluxation occurring in a child after pharyngoplasty. The occurrence of a "spontaneous" subluxation after pharyngoplasty has not been previously reported. The possible scale of neurological complications which may range from relatively minor nerve root compression to a potentially lethal major cord compression emphasises the importance of this condition especially as a minor trauma may precipitate a major disaster. We would like to draw attention to this unusual complication because some neck pain and stiffness are quite usual after pharyngoplasty and we would stress that persisting symptoms associated with a torticollis after any operation in the oral cavity or pharynx should alert the surgeon to this diagnosis. The establishment of early treatment may avert a neurological calamity.

Axis, Cervical Vertebra↗

Localization in human thalamus of units triggered during 'verbal commands,' voluntary movements and tremor.

Microelectrode recordings in the rostral (n. reticularis) and lateral (ventralis lateralis) human thalamus were carried out in locally anaesthetized diskinetic patients during stereotaxic operations. Their responses to voluntary motor tasks prompted by imperative verbal stimuli were tested. Spontaneous and evoked unit activities were studied using computer processing techniques. In the n. reticularis thalami and immediately adjacent thalamic zones, not only units reacting during the initiation of voluntary movements (100-200 msec before the movement), but also units responding to the verbal command itself ('triggered verbal command' units) were found. They proved to be concerned directly with the semantic meaning of the command. In the VL anterior area (Voa-Vop in German nomenclature) the majority of the units responded during the phases of initiation and/or realization of the voluntary motor act ('voluntary movement' units of Jasper and Bertrand 1966); when these units were not spontaneously rhythmic they were transiently transformed into rhythmic (5 +/- 1 Hz) ones. This transformation appeared during the preparation and realization of movement but also in some cells as a rebound phenomenon. In patients without tremor (akinetic and rigid forms of parkinsonism, torticollis), the transient rhythmogenic transformation was frequently provoked by the repetition of motor tasks. In the posterior part of VL (Vim), cells were driven by proprioceptive inflow coming from a specific peripheral region. They react also during the voluntary movement of the same region. The majority of these units were rhythmic at 5 +/- 1 Hz, and they presented a close correlation in phase and frequency with the tremor. The anatomical locations of the three main pools of neurons were determined. 'Triggered verbal command' units were placed more anteriorly and laterally. 'Voluntary movements' and 'rhythmic 5 +/- 1 Hz units' had identical spatial localizations. This fact supports the contributions of these two last types to the central mechanisms of both tremor and voluntary movement.

Electromyography↗

Further evidence for the role of the caudate nucleus in programming motor and nonmotor behavior in Java monkeys.

This study describes the short-term effects of intracaudate microinjections of carbachol in temporarily isolated and restrained Java monkeys. The monkeys were found to display a series of motor disturbances including blepharoptosis, facial twitches, tongue protrusions, ear flattening, torticollis, and compulsive alternations of rapid flexions and extensions of the extremities. In general, carbachol was found to produce consistent effects as far as it concerns its ability to elicit motor disturbances. Three of the five tested monkeys had previously received another series of carbachol injections when they were freely moving and living in a stabilized social group. Accordingly, the present study enabled us to compare the effectiveness of threshold doses of carbachol in the same monkey in two distinct situations. We concluded first, that motor disturbances and disturbances in social communication were closely coupled in relation to the involvement of a particular cholinoceptive substrate within the caudate nucleus of Java monkeys. Second, the motor disturbances under study appeared to require a larger degree of dysfunctioning of this substrate than did subtle disturbances in the social communication of these monkeys. And, finally, stress inherent to restraint increased the susceptibility of the cholinoceptive substrate within the caudate nucleus. The clinical impact of our findings is discussed in view of differences between the premorbid and manifest phases of Parkinson's disease.

Animal Communication↗

Surgery for gastroesophageal reflux in children with normal pH studies.

Esophageal pH monitoring is recognized as the best diagnostic procedure for gastroesophageal reflux (GER) and operation is seldom recommended in the absence of abnormal pH data. To emphasize that operation should not be ruled out for children who may have false-negative pH studies, we report 14 patients operated on for GER in spite of normal pH-monitoring. The mean age was 54 months (range, 18 to 90). Clinical features included vomiting, dysphagia, respiratory disease, anemia, and torticollis. All had radiologic evidence of GER, and 10 had endoscopic and histological esophagitis. Conventional pH-monitoring values were normal but lower esophageal sphincter pressure and propulsive peristalsis were significantly decreased whereas nonpropulsive contractions were predominant. Operation was recommended after an average of 24 months of unsuccessful medical treatment. Independent postoperative assessment showed that 13 of the 14 patients were relieved of their symptoms and dysphagia persists in one. We suggest that the diagnosis of GER should be accepted on the basis of sound clinical judgement plus more than one abnormal test even when pH results are normal. Operation should not be withheld when clinically indicated. There are several explanations for false-negative pH studies, of which alkaline reflux is probably the most important and warrants further investigation in children.

Child, Preschool↗

Congenital skeletal malformations and cleft palate induced in goats by ingestion of Lupinus, Conium and Nicotiana species.

Three piperidine alkaloid containing plants, Conium maculatum (poison-hemlock), Nicotiana glauca (tree tobacco) and Lupinus formosus (lunara lupine), induced multiple congenital contractures (MCC) and palatoschisis in goat kids when their dams were gavaged with the plant during gestation days 30-60. The skeletal abnormalities included fixed extension or flexure of the carpal, tarsal, and fetlock joints, scoliosis, lordosis, torticollis and rib cage abnormalities. Clinical signs of toxicity included those reported in sheep, cattle and pigs--ataxia, incoordination, muscular weakness, prostration and death. One quinolizidine alkaloid containing plant, Lupinus caudatus (tailcup lupine), on the other hand, which is also known to cause MCC in cows, caused only slight signs of toxicity in pregnant goats and no teratogenic effects in their offspring.

Abnormalities, Multiple↗

Avian toxoplasmosis: experimental infection of chicken and pigeon.

Two groups of 13 new-laying hens each were infected by crop-route with 5000 and 50,000 infective oocysts of T. gondii. Four groups of 5 pigeons each were inoculated by crop-route with 50, 500, 1000 and 5000 infective oocysts. To each group of infected birds suitable controls were added. Hens from the experiment with 5000 infective oocysts were apparently resistant to the infection and they had no clinical signs in the succeeding 40 days p.i. Hens from the experiment with 50,000 infective oocysts showed an egg-drop and mortality in embryonated eggs, especially during the first 2 weeks p.i. Isolation of the parasite was unsuccessfully attempted from 720 embryonated eggs, produced by infected groups, and tested on various days p.i. and at different stages of infection. The parasite was isolated from the brain, heart, liver, spleen and lung of infected birds 7 and 15 days p.i.; 40 days p.i. it was evident only in brain and heart. IgG onset and mean course were monitored by ELISA and high titers were reached by both groups. Pigeons from groups 500, 1000 and 5000 developed rapidly progressive clinical signs as diarrhea, trembling, incoordination, torticollis and death. They had enlargement of liver and spleen and focal necrosis, nodular features in the crop. Pigeons from expt 50 had no clinical signs in spite of the presence of the parasite in their organs for over 45 days p.i. Parasite was isolated from brain, heart, liver, spleen, lung, kidney, crop and muscles from all infected groups. Histopathological and ultrastructural features revealed the presence of multiplying tachizoites even within cells of the crop. Seroconversion, as monitored by ELISA, was recorded in all infected groups although high ELISA-titres were never reached. One of the negative controls from expt 5000 developed specific antibodies but the parasite was not isolated from its organs.

Animals↗

Subluxation of the atlanto-axial joint.

A case of post operative atlanto-axial dislocation is presented which was diagnosed on CT scan although the clinical signs and symptoms should have suggested the diagnosis. It is recommended that all patients who present with torticollis in the early post operative period should be considered to have C1-C2 dislocation and an orthopaedic opinion should be sought.

Adolescent↗

Interaction between Eimeria uzura infection and aflatoxicosis in Japanese quail (Coturnix coturnix japonica).

The clinical signs and gross lesions caused by Eimeria uzura (10(5) oocysts) in Japanese quail (Coturnix coturnix japonica) exhibited little or no influence in the face of intercurrent dietary aflatoxicosis (1 p.p.m. of aflatoxin B1 from Day 0 to 55). Similarly, no significant differences in the mucosal morphology of the intestine were evident histologically between the two groups of Japanese quail. The nervous signs of ataxia, leg weakness, incoordination of movement, torticollis and terminal opisthotonos were toxin-induced manifestations. In the aflatoxic quail, hypoplastic changes and selective depletion of lymphocytes were more prominent in the bursa of fabricius. Increased relative mean weights of liver, kidney, spleen, crop, proventriculus and gizzard were observed in birds due to aflatoxin sensitivity. The combination of E. uzura infection and aflatoxicosis in Japanese quail may cause significant weight loss, and increased oocyst production and reproductive potential.

Aflatoxin B1↗