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[Dystonias: what the practitioner needs to know].

Dystonia is characterized by involuntary muscular contractions resulting in repetitive movements and/or postures, with co-contraction of antagonist muscle groups. the present overview describes the fundamentals of dystonic movement disorders, and provides information on their diagnosis and therapy, including in particular the not infrequent focal dystonias spasmodic torticollis, blepharospasm, and writer's cramp, and the generalized forms. For the practitioner it is of primary importance to be able to recognize these sometimes bizarre movement disorders as dystonias. In many cases, the correct diagnosis makes possible effective treatment that frequently results in an appreciable improvement in the patient's symptoms.

Blepharospasm↗

[Sequentially occurred bilateral atlantoaxial rotatory subluxation following craniotomy: a case report].

Atlanto-axial rotatory subluxation (AARS) often presents in childhood as a stiff, painful neck with associated typical cock-robin type of torticollis. It can occur spontaneously or following minor trauma or an inflammatory process in the cervical tissues or following otorhinolaryngeal surgery. We report a case of bilateral AARS which occurred sequentially following craniotomy. To the best of our knowledge, no case of AARS after craniotomy has been reported previously and only one case in which bilateral AARS was observed has been reported to date. Failure to recognize the condition may result in a fixed rotatory subluxation, which can be difficult to treat. Awareness of this condition by surgeons involved in brain surgery should lead to its early recognition and treatment, preventing a fixed deformity occurring. We discuss the clinical presentation and management of AARS. The literature on this subject is briefly reviewed.

Astrocytoma↗

An overlooked association of brachial plexus palsy: diaphragmatic paralysis.

Diaphragmatic paralysis in newborns is related to brachial plexus palsy. It can be overlooked if thorough examination isn't done. We present a two-weeks-old baby with a birth weight of 3800 grams who had a left-sided brachial plexus palsy and torticollis with an undiagnosed left diaphragmatic paralysis even though he was examined by different physicians several times. The role of physical examination, the chest x-rays of patients with brachial paralysis and the treatment modalities of diaphragmatic paralysis due to obstetrical factors are discussed.

Brachial Plexus Neuropathies↗

[Role of the human thalamic parafascicular (CM-Pf) complex in neuronal mechanisms of selective attention].

Responses of 93 single units of the human thalamic CM-Pf complex to relevant and irrelevant verbal (or sensory) stimuli were studied using microelectrode technique in alert diskinetic patients suffering from the tonic forms of spasmodic torticollis during 11 stereotaxic operations. The response patterns of two types units with irregular unitary (A-type) and low-threshold bursting Ca(2+)-dependent (B-type) spike activity were studied. Three main conclusions emerge from the studies: 1) high reactivity of both A- and B-units to presentation of relevant verbal stimuli with differences of their response patterns as determined by the type of constituent elements; 2) close functional connectivity of these neuronal changes with the level of selective attention; 3) at the moment of attention activation, the appearance of transient interneuronal interactions between adjacent A and B cells characterized by the local synchronization and stabilization of rhythmic oscillations. These data point to considerable contribution of the thalamic CM-Pf complex and its neuronal mechanisms into organization of the human selective attention and triggering verbal-related processing during performance of purposive speech-provoked voluntary acts.

Acoustic Stimulation↗

[Cavernous angioma of the cervical spinal cord].

A 34-year old man gradually developed a paraparesis which spontaneously regressed within a few months. One year later, the patient suffered from acute torticollis immediately followed by flaccid tetraparesis with pyramidal signs, dissociated sensory deficit and acute retention of urine. Magnetic resonance imaging (MRI) showed, in the cervical cord, a vascular malformation that was not opacified at angiography. The lesion, a cavernous angioma, was surgically removed, leading to recovery. So far, thirty five cases of spinal cord angioma have been published, most of them since the advent of MRI which makes it possible to determine the exact incidence of this lesion, as well as its signs, symptoms and course.

Adult↗

Grisel's syndrome: a case report.

A 7-year-old girl came to the orthopedic department complaining of neck pain and torticollis following upper respiratory tract infection for 3 days. Roentgenographic examination of the antero-posterior open-mouth view showed type one C1-C2 subluxation. The patient was treated with appropriate antibiotics and cervical traction. A SOMI brace was used for immobilization. The subluxation returned to the normal position after 3 days.

Anti-Bacterial Agents↗

[A new therapeutical approach in neurology (author's transl)].

The therapeutic action of Tiapridal appeared to the authors particularly precious and constant in delirium tremens, senile agitation and turbulence, whatever the origin, and bucco-linguo-facial dyskinesia, whether the latter were linked to age or whether they formed part of a neuroleptic syndrome. Their experience does not permit them to have any opinion concerning the use of this drug in tremor and chorea, the patients seem to respond favourably to treatment but in an erratic manner. On the other hand the drug was totally inefficacious in patients suffering from spasmodic torticollis and writer's cramp. Finally, it seemed to them useful to emphasise the improvement in comfort in patients suffering from various pains when given Tiapridal. This justifies the place given to Tiapridal among drugs necessary for the daily practice of neurology.

Adult↗

[Somatosensory disorders in patients with extrapyramidal pathology: the effect of a stereotaxic intervention].

The authors compared somatosensory evoked potentials (SSEP) recorded in 16 patients with infantile cerebral paralysis (ICP), 16 patients with parkinsonism (P), 18 patients with torticollis (T), and 15 healthy individuals. Structural changes of SSEP were found in all patients with ICP and in most patients with P and T. The structural changes of SSEP in these patients were mainly associated with the absence or suppression of one or two components of SSEP of a healthy individual. It is assumed that one and the same pathological process underlies these three diseases, but it differs in the degree of manifestation in each one. Two groups of patients with parkinsonism which differed in age were distinguished, which represent, probably, two different pathological processes. Certain normalization of the muscular tonus and inhibition of hyperkinesia in most patients as the result of stereotaxic operation was not attended by normalization of the SSEP structure.

Adolescent↗

Jefferson fracture in a 2-year-old child.

Fractures of the atlas vertebra in the pediatric population are very rare, yet must be considered when evaluating a child with neck pain and torticollis. Plain radiographs and computerized tomography should be used in conjunction to confirm the diagnosis.

Cervical Atlas↗

[Oculomotor surgery in Parinaud's syndrome].

The authors report the result of surgery on a case of Parinaud's syndrome with a torticollis chin down of the Alajouanine type (the flexion of the head was used to allow an elevation of the eyes; from this position, depression was possible). A Fadenoperation on both medial recti aimed to suppress the convergence spasms. A resection of both superior recti aimed to substitute for the flexion of the head. The results clearly showed that it is possible to improve the oculo-motricity in a gaze palsy by substituting surgery for the compensatory reflexes. A Fadenoperation which does not destroy the previous parallelism in the primary position is a good indication for any convergence spasm.

Child, Preschool↗

[Calcification of the intervertebral disks in children: apropos of a case].

The authors report a case of calcifications of an intervertebral cervical disk in a 12-year-old boy. A torticollis was the clinically revealing event. As in the majority of the published cases, the etiology remained unknown and the evolution was simple with complete regression of the calcifications.

Calcinosis↗

[Myofascicular hypertonia as a generator of peripheral determinant structure].

The reflex nature of the myofascicular hypertonus (MFH) is evidenced. This is a consequence of the changes in muscular contractile activity. Local, segmental and suprasegmental motor control structures participate in the MFH pathogenesis. MFH generates a perifheral determinant structure. It is capable of substantially changing the manifestations of spastic torticollis++ and syringomyelia. MFH is a major factor of the secondary contracture in the face muscles. It is important in muscular tonic manifestations in spondylogenic neurologic disorders. Postisometric relaxation (manual therapy) provides a means of pathogenetic treatment for MFH.

Contracture↗

Sternomastoid tumor of infancy (fibromatosis colli): diagnosis by aspiration cytology.

Sternomastoid tumor (SMT), as part of the spectrum of muscular torticollis, presents in infants as a discrete mass lesion in the neck, typically unassociated with neck torsion. We describe two patients who underwent fine needle aspiration (FNA) of their neck mass. The cytopathology of these aspirates was composed solely of a mixture of benign-appearing spindle cells and degenerating myofibers. Both infants showed a gradual disappearance of the mass without surgical intervention. We believe that FNA cytology is a diagnostic tool applicable to neonates. Additionally, in the proper clinical setting, aspiration cytology can be diagnostic of the clinicopathologic entity of SMT.

Biopsy, Needle↗

Phenothiazine-induced oculogyric crisis.

Extrapyramidal symptoms such as torticollis, oculogyric crisis and blepharospasm are typical manifestations of phenothiazine-induced drug reactions. Widespread use of such drugs, especially in children make these symptoms relatively commonplace. A case of phenothiazine-induced oculogyric crisis is presented. Included is a discussion of phenothiazine pharmacology.

Adult↗

Juvenile fibromatosis and other diseases of connective tissue.

There are a number of features which are common to such variable conditions as juvenile fibromatosis on the one hand and torticollis and congenital dislocation of the hip, and possibly scoliosis, on the other. Various pathological syndromes are presented, all of them characterized by disturbance and contracture of connective tissue. Surgery is carried out in severe cases when it is a practicable proposition. The administration of an enzyme inhibitor at cellular level has introduced a new dimension in the management of localized fibromatosis.

Birth Injuries↗

Genetics, geography and intelligence in the torsion dystonias.

Personal study of 137 individuals with torsion dystonia (TD) in 92 families, and review of 476 reported cases indicate that there are at least two hereditary forms of TD in addition to acquired dystonia. Autosomal recessive TD, with onset generally between the ages of 4 and 16 and rapid initial course, has been found in highest frequency in the Askenazim. Autosomal dominant dystonia is more variable in its time of onset and course and torticollis is frequently an early symptom. Families with this form are present in several populations. The psychometric performance of patients with autosomal recessive TD is superior to that of controls. In contrast, families in which the dominant form is segregating often contain individuals with below average IQ but this may be a social effect and reflect a limited choice of mates by members of such families.

Adolescent↗

Acute anticholinergic action in focal dystonia.

We have investigated the effects of the intravenous administration of three anticholinergic drugs (atropine, benztropine, and chlorpheniramine) with variable antihistaminic properties in comparison with saline in adult-onset focal dystonias. In many patients, the severity of the involuntary movements varied from one observation to the next. Patients with spasmodic torticollis, writer's cramp, and segmental dystonia showed little change with any of the active drugs. The occasional individual who improved by more than 20% did so at the expense of drug-induced drowsiness. We conclude that cholinergic mechanisms are not of general importance in the pathogenesis of these conditions. However, the beneficial response to benztropine and chlorpheniramine in two of 20 patients emphasizes the continuing need for a trial-and -error approach in the therapy of these focal dystonias.

Acute Disease↗