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"Off" painful dystonia in Parkinson's disease treated with botulinum toxin.

The "off" painful dystonia (OPD), usually concerning the feet, is a type of abnormal involuntary movement, induced by the chronic use of levodopa. It is mostly observed in the advanced stage of Parkinson's disease (PD), particularly in the early morning, in the evening, and late at night. Indeed, some patients have experienced OPD also during "on" periods when dystonic posture of the foot alternates with dyskinesia. The pain probably is due to sustained muscle contraction, which causes prolonged muscle spasm, as in primary dystonia or torticollis. Dopaminergic drugs like bromocriptine, pergolide, and especially apomorphine (s.c. infusions, or bolus), can dramatically improve the OPD. Anticholinergics baclofen and lithium are alos used in the management of OPD with some benefit. On the other hand, clinical experience shows that in many cases, these therapeutic procedures are not always enough to produce the expected results. Thirty PD patients (22 men and eight women) with OPD of the foot were treated with botulinum toxin (Botox, Btx) using electromyograms to guide injections. Dystonia was evaluated using a quantitative rating scale. The selection of the muscles for Btx treatment was carried out on the basis of foot posture. We injected Btx into tibialis posterior, tibialis anterior, gastrocnemius, flexor digitorum longus, and extensores hallucis longus with a median dose 40 IU for each muscle, distributed in two sites. In all patients, the pain improved within 10 days, whereas in 21 patients, the pain disappeared completely for 4 months (range, 3-7 months); a concomitant improvement in intensity of the dystonic spasm was also observed. No side effects were reported. Seven patients with associated "on" foot dystonia described an improvement of foot posture on walking. In conclusion, in this uncontrolled study, the use of Btx in OPD seemed a promising tool to improve pain linked to foot dystonia; however, because of the well-known underlying dopaminergic defect in OPD, the Btx therapy should be considered only if the dopaminergic treatment established for the management of OPD has failed.

Aged↗

Clozapine use in Parkinson's disease: a retrospective analysis of a large multicentered clinical experience.

We conducted a multicentered, retrospective review of clozapine's (CZP) effects on a range of psychiatric, sleep, cognitive, motor, and sensory disorders in Parkinson's disease (PD). Therapeutic outcomes and adverse events were compared with varying prescribing practices at participating sites. The medical records of 172 consecutive PD patients treated with CZP at four movement disorder clinics were reviewed. Low-dose CZP improved psychiatric symptoms of psychosis, anxiety, depression, hypersexuality, sleep disturbance, and akathisia. Tremor, torticollis, limb dystonia, and pain showed modest rates of improvement. Twenty-three percent of patients withdrew as a result of adverse events or treatment failure. Inpatient CZP initiation did not improve therapeutic efficacy, or reduce adverse events or the withdrawal rate. Low-dose CZP in the outpatient setting is generally an effective and well-tolerated treatment for many of the psychiatric, sleep, motor, and sensory disturbances common to late-stage PD.

Aged↗

Comparison of acute- and delayed-onset posttraumatic cervical dystonia.

Head, neck, or shoulder trauma is an occasional antecedent event before the appearance of cervical dystonia. A clinically distinctive syndrome of acute-onset posttraumatic cervical dystonia characterized by markedly restricted range of neck motion, absence of phasic involuntary movements, and poor response to treatment has previously been described. Patients with cervical dystonia attending a movement disorder clinic were reviewed for history of trauma before onset of symptoms. Patients with symptom onset within 4 weeks of trauma were compared with patients who developed symptoms between 3 months and 1 year after trauma. Acute-onset cervical dystonia was characterized by markedly reduced cervical mobility; prominent shoulder elevation with trapezius hypertrophy in most patients, absence of involuntary movements, sensory tricks, or activation maneuvers; and poor response to botulinum toxin injection. By contrast, delayed-onset cervical dystonia was clinically indistinguishable from nontraumatic idiopathic cervical dystonia. Acute-onset posttraumatic cervical dystonia is similar to limb dystonia after peripheral trauma and may represent a form of nondystonic muscle spasm similar to torticollis associated with musculoskeletal injuries of the cervical spine and craniocervical junction.

Acute Disease↗

Myofascial pain mimicking radicular syndromes.

Myofascial pain is very often underscored and misunderstood in clinical practice. In many cases the localization of myofascial pain may resemble other diseases, such as radicular syndromes (e.g., low back pain from herniated disc) and even diseases of internal organs (e.g., angina pectoris, bowel diseases or gynaecological disturbances). In pain clinics one can routinely see patients with myofascial painful disorders showing a radicular topography and normal CT and MRI: as a consequence, when vertebral abnormalities are present on CT or MRI, it should be checked whether the cause of pain is radicular, myofascial, or both. On the other hand, the conventional approach to painful disorders may lead to errors and wrong diagnosis, depending on several factors: a) pain is often considered a symptom of an organic disease; b) the diagnosis is usually directed towards the structural cause of pain only; c) the functional components of the suffering patient are underscored; d) the site of pain may introduce some bias. When the latter is concerned, it is usually admitted that a neck pain may depend on muscle contraction (e.g. torticollis), while such a cause is less commonly admitted for leg, where the attention is first directed towards the sciatic nerve; myofascial origin of pain is even less considered in abdominal or pelvic painful disorders, where patients with no structural detectable diseases are often considered as neurotic and referred to the psychiatrist. The reason for this topographical dependence of diagnosis lies in the conventional attitude to focus on the most relevant and frequent organic diseases, thus introducing a bias with relevant epistemological implications.

Back Pain↗

Stereotaxis and abnormal movements.

A series is presented of 106 patients with extrapyramidal syndromes treated by stereotaxy. The importance of preoperative screening to assure favorable results is stressed. Surgical contraindications are elderly patients with dementia and akinesia. Axial dystonia and spasmodic torticollis respond poorly to stereotaxy; and bilateral interventions should be avoided.

Humans↗

High-frequency cervical spinal cord stimulation in spasticity and motor disorders.

High frequency stimulation of the cervical spinal cord was used in an attempt to moderate motor disorders in 10 patients, 5 spastics, 3 dystonics and 2 with spasmodic torticollis. Through a C4 flavectomy a quadripolar flat electrode was introduced into the epidural space, placing the first terminal at C2. Electrical parameters for stimulation were established at 0.05-0.10 msec pulse width, rate of 200-1,400 Hz and amplitude to paresthesia threshold. Stimulation was delivered 1 hour on and 1 hour off during daytime. Subjective, clinical and/or neurophysiological initial improvement were initially observed in 9 cases, in which the stimulation system was permanently implanted. After a mean follow-up of 41.4 months all patients but one had a clinical and neurophysiological condition almost similar to the prestimulation one. To investigate the effect of the so-called high-frequency stimulation on the nervous system, 9 dogs were implanted following the same surgical technique and stimulated with analogous electrical parameters than in human practice. After follow-up of 8 months the pathological studies of the animals did not show any local or suprasegmental alterations on nervous structures except a local huge dural scarring reaction.

Adult↗

The length-tension diagrams of human oblique muscles in trochlear palsy and strabismus sursoadductorius.

We determined the relation between length and tension in detached oblique muscles of 16 strabismus patients that underwent surgery, before and during contraction evoked by intravenous administration of succinylcholine. We frequently found a nonlinear relation between length and tension, unlike our previous findings in recti. In superior oblique palsies, the superior oblique was found, before injection of succinylcholine, to be stiff after elongation, and did not contract after injection of succinylcholine, while the ipsilateral inferior oblique contract after injection of succinylcholine, but with a higher spring constant than did usual. In 3 cases the superior oblique contracted vividly after administration of succinylcholine despite the presence of excyclotropia, stereopsis, torticollis (2 cases) and a hypertropia that increased in adduction, in downgaze, in adduction-and-downgaze and on ipsilateral head-tilt. The finding of a vividly contracting superior oblique is incompatible with the diagnosis of a complete superior oblique palsy. We conclude that some of the cases diagnosed as congenital superior oblique palsy, having a hypertropia increasing in adduction, in downgaze, in adduction-and-downgaze and on ipsilateral head-tilt, are in fact cases of unilateral strabismus sursoadductorius (upshoot in adduction), a non-paretic motility disorder.

Adolescent↗

Cervical intervertebral disc calcification in children.

Intervertebral disc calcification in children is a rare occurrence. The clinical symptoms and signs are distinctively confined to the cervical area with pain, limitation of motion, and torticollis. Long tract signs or radicular involvement are extremely unusual. CT scan and cervical spinal X-ray films shown the calcification to be in the nucleus pulposus with anterior or posterior mild protrusion into the spinal canal. Recovery without neurological sequelae is the rule in most of the pediatric cases with conservative treatment. Intervertebral disc calcification does not necessarily disappear with the onset of clinical symptoms. A case with persistent and even denser calcification at the same level of intervertebral disc space at the second episode of recurrence is illustrated.

Calcinosis↗

Benign congenital muscular dystrophy with autosomal dominant heredity: problems of classification.

A family with autosomal dominant congenital muscular dystrophy affecting members of both sexes in three generations is described; a father and his two sons were studied. The onset of symptoms was in early childhood and progression, if any, was slow. The proximal limb muscles, the sternocleidomastoid and anterior tibial muscles were affected. One patient had torticollis and all had heel-cord shortening. An electrophysiological examination showed myopathy. There was no cardiomyopathy. Creatine kinase (CK) was elevated, and a histological study revealed a necrotizing myopathy with pronounced regeneration and formation of aberrant myofibrils (ringbinden) and fibrosis.

Adolescent↗

[Idiopathic blepharospasm. Clinical and electrophysiological investigations in 27 patients (author's transl)].

Idiopathic blepharospasm is reexamined and the differential diagnosis discussed on the basis of the signs and symptoms of 27 unpublished cases. As in tardive dyskinesia, middle-aged and elderly women are mostly affected. Contrary to general opinion, spontaneous improvement occurred in about one-fifth of the patients. The habituation of the orbicularis oculi reflexes (investigated electromyographically) was diminished in 52% of the patients. These and other symptoms indicate an organic cerebral lesion as the main cause of the disease. Similarities existing between blepharospasm and torticollis spasticus are discussed.

Basal Ganglia Diseases↗

Oropharyngeal dysphagia due to iatrogenic neurological dysfunction.

Oropharyngeal dysphagia due to iatrogenic neurological dysfunction may relate to either medication side effects or surgical complications. There are several general mechanisms by which neurological side effects of medications can cause or aggravate oropharyngeal dysphagia. These include decreased level of arousal, direct suppression of brainstem swallowing regulation, movement disorders (dyskinesias, dystonias, and parkinsonism), neuromuscular junction blockade, myopathy, oropharyngeal sensory impairment, and disturbance of salivation. Postsurgical oropharyngeal dysphagia due to neurological dysfunction has been described in association with carotid endarterectomy, esophageal cancer surgery, anterior cervical fusion, and ventral rhizotomy for spasmodic torticollis. A potential explanation for oropharyngeal dysphagia following these surgical procedures is intraoperative mechanical disruption of the innervation of the pharyngeal constrictor muscles by the pharyngeal plexus. Posterior fossa and skull base surgery can lead to dysphagia as a result of intraoperative damage to brainstem centers and/or cranial nerves involved in swallowing. Perioperative stroke is the most likely explanation for oropharyngeal dysphagia appearing acutely following surgery, especially if the type of surgery predisposes to embolism or hypoperfusion.

Anticonvulsants↗

Clinicoradiological diagnosis of benign osteoblastoma of the spine in children.

Three cases of benign osteoblastoma of the spine in children are reported, diagnosed on the basis of clinicoradiological findings and confirmed at surgery and by histopathology. The authors feel that in all cases of painful scoliosis and painful torticollis, a careful search should be made for any suspicious area of sclerosis, especially in the posterior elements of the vertebrae, and all the positive or suspected cases should undergo nuclear bone scanning. All patients whose bone scan is positive should be subjected to computed tomography for differential diagnosis between osteoid osteoma and benign osteoblastoma.

Adolescent↗

The acetylcholine-dopamine balance in the basal ganglia of rhesus monkeys and its role in dynamic, dystonic, dyskinetic, and epileptoid motor activities.

Behavioural analyses have been made of effects brought about by both single and combined injections of dopamine, haloperidol, carbachol and atropine into the caput nuclei caudati of rhesus monkeys. High doses of dopamine produced the subsequent development of three types of behavioural changes: an increase in the number of skilled manipulation movements (the dynamic phase); the appearance of a dystonic torticollis (the dystonic phase); and, finally, the appearance of an oro-lingual-facial dyskinesia and a number of dyskinetic activities in the extremities (the dyskinetic phase); low doses of dopamine solely produced the dynamic phase. Haloperidol only inhibited the dopamine-induced dynamic and dystonic phase: it did not suppress the dyskinetic phase. High doses of carbachol produced the subsequent development of four phases: a dynamic, dystonic, dyskinetic and epileptoid phase; the last one was marked by the appearance of secondary generalized epileptic seizures. Low doses of carbachol solely produced the dynamic phase. Atropine inhibited the carbachol-induced dynamic, dystonic and epileptoid phase; it did not suppress the dyskinetic phase. High doses of dopamine strongly modified the carbachol-induced phases: dopamine intensified the dystonic phase on the one hand, but it abolished the generalized epileptic seizures on the other hand. Apart from the fact that the data presented have confirmed that both dopaminergic and cholinergic mechanisms within the basal ganglia of rhesus monkeys are involved in the elicitation and modulation of both normal and abnormal motor activities, they have also revealed that the simple concept of a stristal acetylcholine-dopamine "see-saw" has to be revised. Furthermore, the data have suggested that development of supersensitive dopamine-sensitive sites is not the only mechanism that underlies the elicitation of the oro-lingual-facial dyskinesia. And finally, the present experiments have given clearcut evidence that an intrastriatal acetylcholine-dopamine "see-saw" fulfils and essential role in the process involved in the generalization of epileptic seizures.

Acetylcholine↗

Case report: concordant traumatic brainstem contusion delayed diagnosis in a young man with Wilson's disease.

Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism. The corresponding gene locus has been localized on the long arm of chromosome 13. Three different clinical variants of the disease can be distinguished: hepato-cerebral, abdominal/hepatic, and central nervous type. The heterogeneity of symptoms can cause problems in differential diagnosis, especially when another concordant disorder can also explain the pathogenesis of symptoms. The case report of a young man who suffered from brainstem contusion demonstrates the possibilities of misinterpretation because presenting symptoms could be attributed either to traumatic brain injury followed by adjustment disorder or Wilson's disease. Clinical signs included leftsided hemiparesis, bilateral gaze direction nystagmus, marked dysarthria with consecutive pervasive mutism, choreo-athetoid movements, spasmodic torticollis and diplopia dependent on gaze direction. Slit lamp examination showed Kayser-Fleischer's corneal ring. EEG- and computer assisted tomography investigations revealed non-specific findings. The patient was treated with D-Penicillamine. Alternative treatment with oral zinc preparations is discussed.

Adolescent↗

Intracranial chordoma in a neonate.

We report the first case of a congenital intracranial chordoma. Hydrocephalus, sixth and seventh cranial nerve palsy, and torticollis were observed shortly after birth. The tumour was delineated by sonography, CT scans and MRI and the diagnosis confirmed after subtotal resection at the end of the newborn period.

Abducens Nerve↗

A four-step test for diagnosis of pseudo superior oblique palsy.

An 8-year-old boy presented with an ocular torticollis that had appeared many months after a ptosis repair. The three-step test was positive for a superior oblique palsy. However, at the time of surgery the forced duction test showed a marked restriction in depression of the eye. These findings were duplicated before the second procedure normalized the ocular movements. The surgical microscope was of great help for the extensive dissection necessary to correct the condition. The fourth step, the forced duction test, was essential for accurate differential diagnosis between true superior oblique palsy and a mechanical hypertropia.

Child↗

Management of superior oblique overaction in A-pattern deviations.

Surgical management of overaction of the superior oblique muscle is discussed with reference to such conditions as A-pattern, Brown's syndrome, torsional torticollis, and homolateral inferior oblique or contralateral inferior rectus paresis. The authors perform weakening of the superior oblique from the temporal side of the superior rectus for slight or moderate overaction. They prefer posterior tenectomy for mild overaction and scleral disinsertion of the tendon in moderate overaction causing A-pattern of no more than 25 pd. With 25-30 pd. they perform translation-recession of the muscle by the nasal approach.

Esotropia↗

Osteoid osteoma of the axis.

A 15-year-old boy suffering from osteoid osteoma of the right lamina of the axis presented with torticollis and local pain without neurological deficits. The tumour was treated by en-bloc resection and laminoplasty with autogenous iliac crest. The follow-up at 14 months showed complete pain relief and a perfect incorporation of the transplanted bone.

Adolescent↗