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Intrathecal baclofen therapy for stiff-man syndrome and progressive encephalomyelopathy with rigidity and myoclonus.

We report on eight patients with stiff-man syndrome (SMS) or its "plus" variant, progressive encephalomyelopathy with rigidity and myoclonus (PERM) receiving intrathecal baclofen via pump. In six of the patients, follow-ups continued for approximately 2.5 to 6.5 years after pump implantation. Intrathecal baclofen was an effective last-resort alternative for patients who responded poorly to or did not tolerate oral antispasticity medications. General mobility increased, and spasms and rigidity were reduced; however, no complete remissions were observed either before or after pump implantation. PERM patients showed more severe and rapid progression of symptoms and more attacks of autonomic dysregulation than SMS patients. They also required higher doses and more rapid dosage increases. Complications of intrathecal baclofen therapy included spasm-induced rupture of the catheter, catheter dislocation causing radicular symptoms, and pump malfunction resulting in inaccurate dosage administration. Patients suffered fewer side effects with intrathecal baclofen than with oral medication, but overdose resulted in a transient, comalike state in one patient and sudden dosage reduction due to pump failure was fatal in another.

Adult↗

Effects of barbiturates on the decerebrate rigidity in rats.

Effects of barbiturates (phenobarbital, mephobarbital, pentobarbital, hexobarbital and amobarbital) on the tonic and phasic responses of forelimbs were studied in the rats in which rigidity was induced by means of anemic decerebration. The inhibitory pattern of the tonic and phasic responses to phenobarbital was different from those to the other barbiturates, i.e. phenobarbital gradually inhibited both tonic and phasic responses, whereas other barbiturates suppressed significantly only the tonic response immediately after the administration of a relatively low dose (10 mg/kg) but hardly inhibited the phasic response unless at higher doses (20 and 40 mg/kg). Possible involvement of GABA-system in the inhibitory action of phenobarbital and pentobarbital on the rigidity was investigated. Since inhibition of the phasic response to pentobarbital was potentiated by the pretreatment of animals with carboxymethoxylamine hemihydrochloride (AOAA) and suppressed by the pretreatment with semicarbazide, it was suggested that GABAergic mechanism may be involved in the particular inhibition of phasic response to pentobarbital. However, the inhibitory action of phenobarbital appeared to be independent of GABA-system.

Aminooxyacetic Acid↗

Neuroleptic malignant syndrome: two cases without muscle rigidity.

OBJECTIVE: Two patients with neuroleptic malignant syndrome without muscle rigidity are described. CLINICAL PICTURE: Both patients developed fever and altered consciousness while taking neuroleptic but did not develop muscle rigidity; the symptoms subsided when the neuroleptic was stopped but recurred when it was given again. TREATMENT: The neuroleptic was stopped; one patient received supportive treatment and the other received bromocriptine. OUTCOME: One patient died while the other survived. CONCLUSION: The pathophysiology is proposed as a combination of involvement of the central thermoregulatory, neuroregulatory and autonomic nervous systems, and the peripheral skeletal muscle. It supports the concept of a spectrum of clinical severity of neuroleptic malignant syndrome.

Adult↗

[Nuchal rigidity in children: meningitis or not?].

Three children, two girls aged 4 and 2.5 years and one boy aged 8 years, presented with nuchal rigidity and symptoms such as fever, headache and nausea. Upon investigation they had: torticollis on the bases of an upper respiratory tract infection, viral meningitis and bacterial meningitis (meningococcus type C) respectively. They all recovered well after treatment. Nuchal rigidity can be caused by many illnesses other than bacterial meningitis. Lumbar puncture should be performed when meningeal irritation is suspected. In children this can be identified using the Vincent test as well as the Kernig and Brudzinski tests.

Child↗

[Unilateral abolition of parkinsonian rigidity after subthalamic nucleus hemorrhage].

A 63-year-old man with parkinsonism suddenly developed a right hemiballism, and the CT showed a hematoma of the left subthalamic nucleus. After the ballistic movement had disappeared, muscular rigidity improved on the right. This case suggests that excessive output from the subthalamic nucleus to the internal segment of globus pallidus plays a critical role in the pathophysiology of parkinsonian rigidity.

Cerebral Hemorrhage↗

[Masseter muscle rigidity after suxamethonium during induction and postoperative abortive malignant hyperthermia in a patient with esophageal achalasia].

A 38-year-old man diagnosed as esophageal achalasia developed masseter muscle rigidity after intravenous suxamethonium during anesthetic induction. Anesthesia was maintained with intravenous agents and epidural blockade, while the masseter muscle rigidity continued. After the surgery, his body temperature increased to 38.8 degrees C concomitantly with the appearance of myoglobinuria suggesting the occurrence of abortive malignant hyperthermia. These symptoms were dissolved by dantrolene administration. He was later proved to be negative with CICR test.

Adult↗

[Rigidity abolished by intravenous dantrolene in a patient with Parkinson's disease under spinal anesthesia].

Spinal anesthesia was given to a patient with right femur fracture and Parkinson's disease (PD). Although sufficient analgesia was obtained up to L1 level after spinal anesthesia, the muscular rigidity remained. Furthermore, tremor of the upper extremities continued. After administering dantrolene sodium (DT) intravenously, these untoward features were abolished. These findings suggest that DT abolishes rigidity and tremor in PD, and is useful for the management of anesthesia for a patient with PD.

Aged↗

[A 67-year-old man with rt. hand resting tremor and rigidity after lt. putaminal hemorrhage].

We report a 67-year-old man with rt. hand resting tremor and rigidity after lt. putaminal hemorrhage. He had hypertension and alcoholic liver cirrhosis as past history. When he was 62 years old, he realized rt. hemiplegia suddenly and admitted in Juntendo Urayasu hospital. Brain CT showed intracranial hemorrhage in lt. putamen. He was treated with neurosurgery operation for rejecting hemorrhage. Mild rt. hemiparesis remained but he could live independently. He was medicated sulpiride for depression after cerebrovascular accident. On 63 years old, resting tremor and rigidity appeared on his rt. hand. His doctor stopped sulpiride and treated with L-Dopa/Benserazide and trihexiphenidyl. His parkinsonism was stable with this treatment for four years. His doctor considered that he was Parkinson's disease or drug-induced parkinsonism. On 67 years old, he became akinetic-mutism state suddenly and admitted in the hospital. His consciousness turned alert soon and discharged after two weeks. This episode was considered as epilepsy. After one week from discharge, he was found cardio-pulmonary arrest and confirmed dead in the hospital. Post-mortem examination revealed necrosis in the posterior-lateral part of lt. putamen due to hemorrhage. However, there was no degenerative change of the striatum or the substantia nigra and no Lewy bodies in his brain. Other pathological changes were also not found. His parkinsonism might be caused putaminal pathology due to hemorrhage.

Aged↗

[Etiology of the rigidity of axial muscles of the trunk in Moersh-Woltman syndrome].

A woman patient suffering from the rigid man syndrome (RMS) is described. Analyzing the clinical characteristics of the case, the anatomophysiological features of segmental innervation and suprasegmental control of the activity of axial muscles of the body, the authors suggest that the phenomenon of axial muscle rigidity is caused by the hyperreactivity of vestibular and reticular neurons stimulating via vestibulo- and reticulospinal fibers the motoneurons of the medial plates of the anterior cornua of the spinal cord (the segmental representation of axial muscles). The pathogenetic similarity of myotonic disorders in axial muscles in patients with the RMS and reflex manifestations of vertebral osteochondrosis is under discussion.

Adult↗

Some pathophysiological aspects of the parkinsonian rigidity.

The neurophysiological mechanisms explaining parkinsonian rigidity are still poorly understood. Its reflex nature is well established but the peripheral afferents causing it are likely multiple and not restricted to IA afferents. Few modifications appear in spinal cord reflex mechanisms and are limited to some interneurones (reciprocal inhibition and flexor reflex). At present, the most plausible explanation of rigidity relies on hyperactivity in long loop reflex pathways relaying in the brain.

Electromyography↗

Prognosis of traumatic decerebrated rigidity.

91 cases with traumatic decerebrated rigidity are reported and compared with age of patients, kind of lesion, time of decerebration. The Authors consider the reversibility of some of these comas with decerebrated rigidity and try to establish some immediate criteria for prognosis, and, in the long term, the quality of the functional recovery achieved.

Adolescent↗

[Effects of diazepam and baclofen on the anemic decerebrate rigidity in rats (author's transl)].

Effects of diazepam and baclofen on the anemic decerebrate rigidity in rats were studied by using the drugs that modify GABAergic mechanisms of deplete catecholamine. Rigid forelimb tension in anemic decerebrate rats took the form of tonically sustained tension (tonic component), while the phasic tension (phasic component) was induced by mechanical stimulation of hindlimbs. Diazepam exerted a marked dose-dependent inhibition of the phasic component, whereas baclofen produced a similar effect on both tonic and phasic components. In rats pretreated with semicarbazide, the inhibitory effect of diazepam on phasic component was reduced, and the effect of semicarbazide was antagonized by pyridoxine. The effect of diazepam was slightly enhanced by pretreatment with aminooxyacetic acid, and antagonized by picrotoxin. In contrast to diazepam, the depressant actions of baclofen on both components were not affected by semicarbazide, aminooxyacetic acid or picrotoxin. Catecholamine depletion produced by alpha-methyltyrosine or disulfiram significantly reduced the effect of baclofen on the phasic component. It thus appears that GABA is involved in the effect of diazepam on the phasic component, and noradrenaline is involved in the same effect of baclofen in anemic decerebrate rats.

Aminooxyacetic Acid↗

Action of lisuride on reserpine-induced muscular rigidity in rats after local application into the striatum, ventricular space or the spinal subarachnoid space.

Muscular rigidity was induced in rats by reserpine (10 mg/kg) and the tonic activity of the gastrocnemic muscle was recorded in the electromyogram. Systemic administration of lisuride, an ergoline, resulted in a dose-dependent depression of rigidity. To examine the site of action of lisuride, we injected lisuride into the striatum, the ventricular space, and the spinal subarachnoid space of intact reserpinized rats. Lisuride is effective if injected into either the striatum or the spinal subarachnoid space, the spinal effect being more pronounced. These results suggest that the spinal cord is an important site of action of lisuride.

Animals↗

[A case of progressive supranuclear palsy showing improvement of rigidity, nuchal dystonia and autonomic failure with trazodone].

A 63-year-old man was admitted to the hospital with a 1,5-year history of progressive dementia, supranuclear ophthalmoplegia, pseudobulbar palsy, rigidity and dystonia in the neck and the upper trunk. Magnetic resonance imagings showed severe atrophy of the frontal lobe and the brainstem. He was diagnosed as having progressive supranuclear palsy (PSP). Rigidity, nuchal dystonia, frequent micturition, and profuse sweating ameliorated after trazodone administration. Furthermore, additional administration of L-dopa and droxidopa improved his pseudobulbar palsy, akinesia, and lack of initiative. Single photon emission tomography using IMP after medication showed increased IMP-uptake in the frontal areas and the basal ganglia compared with that before medication. This patient illustrates a substantial role of impairments in the serotonin system in the production of some PSP symptoms.

Autonomic Nervous System Diseases↗

[Freeman-Sheldon syndrome: generalized muscular rigidity after anesthetic induction].

We describe a case of generalized muscle rigidity in a 2-year-old patient with Freeman-Sheldon syndrome undergoing surgery for eventration of the diaphragm after anesthetic induction with halothane and succinylcholine. Anesthetic induction was by mask with oxygen, nitrous oxide and halothane, with succinylcholine as a muscle relaxant. Approximately 10 minutes after start of induction, muscular rigidity appeared and developed rapidly, becoming severe and compromising ventilation. Tracheal intubation was attempted without success, owing to stiffness of the masseter muscles. Sodium dantrolene 2.5 mg/kg was administered and relaxation was achieved immediately for both masseter and peripheral muscles, such that the patient recovered spontaneous breathing. We conclude that there is risk of association between Freeman-Sheldon syndrome and malignant hyperthermia.

Abnormalities, Multiple↗

[An unusual case of peroneal muscular atrophy with rigidity, polyneuropathy, mental retardation, and diabetes mellitus developed in familial Parkinson's disease].

Familial polyneuropathy mimicking Charcot-Marie-Tooth disease associated with parkinsonism and dementia has been reported in literature. We present with similar peroneal muscular atrophy, rigidity of upper extremities, severe peripheral neuropathy, mental retardation and diabetes mellitus. The patient, a 42-year-old man, developed progressive muscle weakness, mental retardation and difficulty in walking in childhood. Because of his pes cavus, he had three surgical operations. At the age of 20 years, he developed distal muscular atrophy of lower limbs. On neurological examination, all limb muscles were atrophic, especially in lower one third of the thigh. Rigidity was noted in the upper extremities. Deep tendon reflexes were hyperactive in the upper and diminished in the lower extremities. Muscle CT revealed low density areas in all the muscles examined, specially in the gastrocnemius and anterior tibial muscles. Needle EMG showed neurogenic change in the forearm, but not in the lower limbs, because of no voluntary contractions obtained due to severe muscle atrophy. Marked slowing of motor conduction velocity with muscle action potentials of very low amplitude was found in the ulnar nerve. Muscle action potentials were not elicited in the median and peroneal nerves. Sensory action potentials were not elicited from the median, ulnar and sural nerves. These findings were consistent with axonal polyneuropathy. In the sural nerve biopsy, the densities of myelinated fibers were markedly decreased. However, unmyelinated fiber densities were relatively preserved. Onion bulb formation was not found. This patient may be classified into hereditary motor-sensory neuropathy (HMSN) type II based on the clinical findings delayed nerve conduction velocities and axonal degeneration in the sural nerve. He has also diabetes mellitus. CT of the brain revealed nothing particular. He is one of members with familial Parkinson's disease (PD) developed in Sagamihara. Peroneal muscular atrophies are not necessarily associated with PD, though it has been occasionally complicated in various neuro-degenerative diseases including parkinsonism. We are now following the patient to detect the symptom of Parkinson's disease for early treatment.

Adult↗

Quantification of rigidity and tremor activity in rats by using a new device and its validation by different classes of drugs.

Subjective techniques employed for measuring skeletal muscle tone and tremor, leading symptoms of several diseases, have certain limitations. Objective methods are usually more sensitive and accurate. The equipment developed by the authors allows the objective and rapid measurement of experimentally induced rigidity and tremor in the same small laboratory animal (rat). The present method considerably reduces the number of animals needed to investigate the activity of drugs, especially when compounds should be screened. Due to the greater sensitivity of the equipment, doses of reserpine and oxotremorine which do not cause any postural, autonomic or parasympathetic symptoms can be used to induce muscular rigidity and tremor. Therefore, not only the number of animals but also their stress can be reduced. It was possible to differentiate the oxotremorine-induced tremor from the spontaneous motor activity and to determine qualitative differences in tremor caused by antitremor agents. A number of clinically effective muscle relaxants and antiparkinsonian drugs were examined in this model in order to determine its utility.

Analgesics, Opioid↗

Internal carotid artery dissection following rigid esophagoscopy.

A case of internal carotid artery dissection that developed after rigid esophagoscopy is described. The diagnosis was suggested by the clinical presentation and confirmed by the findings of radiological examinations. Internal carotid artery dissection is a rare condition of controversial etiology. Most frequently, the cause is unknown and the condition is termed idiopathic. A few cases have occurred after forceful cervical extensions and manipulations. The pathogenesis in our case is uncertain: while the rigid esophagoscopy is the most probable cause, the intubation and spontaneous carotid artery dissection cannot be ruled out.

Adult↗