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Function and innervation of the involuntary m. retroauricularis.

Beside the automatic, obligatory and tonic coinnervation of the involuntary m. retroauricularis in conjugate lateral gaze (oculoauricular phenomenon, nystagmus) several other physiological ways of accidental coinnervation are described. In talking, chewing, swallowing and during involuntary inspiration irregular bursts of innervation may be registered. In sleep regular rhythmic inspiratory innervation is demonstrated as well as myoclonic jerks. With reservation, an allusion is made to rem-sleep. In "nervous subjects" irregular involuntary innervation of the m. retroauricularis might serve as a measurement instrument for the involuntary somatomotor nervous system, i.e. the degree of neurotic tensity. An early myasthenic reaction is gained from the M. retroauricularis in patients with ocular forms of the disease. A common motor nucleus of abducens and facial nerve is discussed. Complementary studies are announced on the various forms of facial paralysis, strabismus and nystagmus. A further diagnostic use is presumed.

Abducens Nerve↗

[A case of acute renal failure caused by rhabdomyolysis with thrombosis of the deep vein of the right leg, following sleeping in a straight sitting position for a long time].

A 41-year-old woman took an overdose of sedatives on the 13th of January, 1994 and remained a sleep in the straight sitting position until she was woken up on the 15th. The next day she consulted our hospital complaining of pain and swelling of her right leg. X-ray examination with contrast medium revealed obstruction of the deep vein of her right leg by a thrombus. On the 17th, her serum urea nitrogen was 75.9 mg/dl and creatinine was 5.4 mg/dl accompanied by oliguria. The myoglobin value was 27,000 ng/ml in serum and 88,000 ng/ml in urine. She was diagnosed as acute renal failure caused by rhabdomyolysis and hemodialysis therapy was started. She was released from hemodialysis on the 31th of January. The swelling of her right leg disappeared at the end of February. However, her right foot was affected paralysis of the fibular nerve. Electromyogram of her right anterotibial muscle and the test of conduction velocity of right tibial nerve revealed that the neurological disturbance of her right leg was caused by thrombosis of the deep vein. Generally speaking, the swelling of the extremities resulting from rhabdomyolysis caused by crush syndrome is due to a massive shift of body-fluid into the crushed muscles. We believe that when the extremities are compressed (and/or crushed) for a long time, venous thrombosis of the extremities occurs due to compression, there by causing swelling of the compressed extremities, as in this case.

Acute Kidney Injury↗

alpha2 adrenoceptors are involved in the regulation of the gripping-induced immobility episodes in taiep rats.

In 1989 Holmgren et al. (Holmgren et al. 1989 Lab Anim Sci 39:226-228) described a new mutant rat that developed a progressive motor disturbance during its lifespan. The syndrome is characterized by a tremor in the hind limbs followed by ataxia, episodes of tonic immobility, epilepsy, and paralysis. The acronym of these symptoms (taiep) became the name of this autosomic, recessive mutant rat. The taiep rats are neurological mutant animals with a hypomyelination, followed by a progressive demyelination process. At 7-8 months of age, taiep rats develop immobility episodes (IEs) characterized by a cortical desynchronization, associated with the theta rhythm in the hippocampus and changes of the nucal electromyogram (EMG), whose pattern is like rapid-eye-movement (REM) sleep. These rats also show an altered sleep pattern with an equal REM sleep distribution. This study analyzed therole of alpha(2) adrenoceptors in the expression of gripping-induced IEs in 8-month-old male taiep rats. The alpha(2) adrenoceptor agonists clonidine and xylacine increased the frequency of gripping-induced IEs whereas the alpha(2) antagonists yohimbine and idazoxandecreased or prevented such episodes. These findings correlate with the pharmacological observations in narcoleptic dogs and humans in which alpha(2) adrenergic mechanisms are involved in the modulation of cataplexy. Unexpectedly, the repetitive administration of clonidine resulted in jumping behavior, indicative of phasic activation of extensor musculature. Taken together, our results show that alpha(2) adrenoceptors are involved in the modulation in gripping-induced IEs and after the administration of several doses of clonidine produced phasic motor activation.

Adrenergic alpha-2 Receptor Agonists↗

[BiPAP therapy of respiratory disorders in patients with congenital neuromuscular diseases].

BACKGROUND: Patients with hereditary generalized neuromuscular diseases develop respiratory failure and decreased maximal inspiratory pressure (Pi max) due to both, involvement of respiratory muscles in the disease and secondary spine- and thorax-deformity. In recent years mechanical respiratory support was done mostly by hyperbaric pressure ventilation when the patients become hypercapnic during daytime. However, polysomnographic investigations have shown that despite normal ventilation during daytime, severe respiratory failure might be detectable only during sleep. PATIENTS AND METHODS: Fourty patients with hereditary generalized neuromuscular diseases were analyzed using polysomnographic analyses and lung function tests (4-channel-ECG,2-channel-ECG, EOG, respiratory parameters, continuous oxygen saturation measurement and capillary blood gas analyses during sleep). Patients with need of ventilatory support during sleep were treated using bilevel positive airway pressure ventilation (BiPAP). RESULTS: Twenty patients revealed severe sleep related breathing disorders and were therefore treated by BiPAP. All showed normalisation or substantial improvement during BiPAP-therapy except two patients with persistent daytime symptoms. During follow-up (6 to 38 months) 3 patients died (cardiomyopathy, pulmonary embolism, pneumonia). The most important side effect of BiPAP-therapy was pressure marks due to the masks. CONCLUSIONS: BiPAP is useful for treatment of sleep related respiratory failure in patients with hereditary generalized neuromuscular diseases.

Adolescent↗

[Polysomnographic as a therapeutic aid in of psychogenic-functioning paraplegia].

The case of a patient with complete sensory and motor paraplegia for more than 1 year is presented. Leg movements were documented during sleep by video and electromyographic recording because a psychogenic cause of the symptoms was suspected. We showed the video to the patient, which illustrated that leg movements were possible. This resulted in fast and complete resolution of the neurologic symptoms. The patient has now been free of them for more than 3 years. This example suggests that the achievement of consciousness of normal motor function is a therapeutic approach for long-standing improvement of psychogenic paralysis.

Adult↗

The Stockholm spinal cord injury study: 1. Medical problems in a regional SCI population.

Out of a regional traumatic spinal cord injury population consisting of 379 individuals, 353 (93.1%) participated in the present study. Subjects were individually interviewed using semi-structured protocols. In addition, previous medical records were available for over 96% of subjects, and were used in all these cases to minimise recall bias. Cause of injury, prevalence of present medical symptoms and occurrence of medical complications in the post-acute, post-discharge phase were recorded. Neurological classification was verified by physical examination according to ASIA/IMSOP standards. Many subjects had experienced complications since discharge from initial hospitalisation, especially urinary tract infections, decubitus ulcers, urolithiasis, and neurological deterioration. Prevalence of medical symptoms was also high. More than 41% of subjects with spastic paralysis reported excessive spasticity to be associated with additional functional impairment and/or pain. Almost two-thirds of subjects reported significant pain, with a predominance of neurogenic-type pain. Bladder and bowel dysfunction were each rated by nearly 41% of subjects as a moderate to severe life problem. As expected, sexual dysfunction was also commonly reported. Prevalence of reported symptoms by general systems review was high, particularly fatigue, constipation, ankle oedema, joint and muscle problems, and disturbed sleep. However, lack of adequate normative data precludes comparison with the general population. The frequent occurrence of reported medical problems and complications support advocacy of comprehensive, life-long care for SCI patients. The commonly reported problems of neurogenic pain and neurological deterioration, in particular, require more attention, as these symptoms are not seldom ominous, either by virtue of their impact on quality of life, or because of underlying pathology.

Adolescent↗

Conditions associated with canine hypothyroidism.

Careful review of the literature regarding clinical signs caused by hypothyroidism in dogs has shown that some assumptions regarding the relation of hypothyroidism to other conditions are based on anecdotal evidence. Cutaneous manifestations are present in most hypothyroid dogs, but the specific abnormalities and breed variations remain to be clearly defined. Decreased metabolic rate manifested by obesity and lethargy is also common. Neurologic manifestations, although uncommon, clearly occur in hypothyroid dogs. Cardiac abnormalities seem to be common, but their clinical significance is questionable. The only consistent hematologic abnormality that occurs in hypothyroid dogs is anemia; evidence for acquired von Willebrand's disease or other bleeding disorders is negligible. Reproductive dysfunction secondary to hypothyroidism is unlikely to occur in male dogs, and there is no evidence to support abnormalities in female dogs. The relation of megaesophagus, laryngeal paralysis, ocular abnormalities, and gastrointestinal disorders with hypothyroidism remains to be established. Future research into canine hypothyroidism may serve to convert dogma into a more clear understanding of the manifestations and pathophysiologic findings of this common endocrinopathy.

Anemia↗

Shoulder pain in female wheelchair basketball players.

STUDY DESIGN: Descriptive self-report survey. OBJECTIVES: To assess activity level, medical history, and the prevalence and intensity of shoulder and upper extremity pain experienced during functional activities in female athletes who compete in wheelchairs. BACKGROUND: Previous studies have documented a high incidence of upper extremity soft tissue disorders in athletes who compete in wheelchairs. None of these studies have specifically focused on female athletes who use wheelchairs. METHODS AND MEASURES: Forty-six female wheelchair basketball players completed an anonymous survey that included demographic data, medical history data, and the Wheelchair User's Shoulder Pain Index (WUSPI). The WUSPI is a valid and reliable self-report measure scored from 0 to 150, with higher scores indicating a greater intensity of shoulder pain during functional activities. RESULTS: The average age of the respondents was 33.2 (+/- 9.1) years, with an average of 12.5 (+/- 10.2) years of wheelchair use. Their disabilities included 39% spinal cord injury, 28% various lower extremity musculoskeletal and neuromuscular disabilities, 13% postpolio paralysis, 11% spina bifida, and 9% amputations. Only 14% of the subjects reported shoulder pain prior to wheelchair use. In contrast, 72% of the subjects reported shoulder pain since wheelchair use, with 52% reporting current shoulder pain. Overall, the subjects scored an average +/- SD performance-corrected total WUSPI score of 15.6 +/- 20.5 on a scale of 0 to 150 points, with 0 representing no pain. The highest intensity of shoulder pain was reported during household chores, propulsion on ramps or inclines, lifting overhead, and while sleeping. CONCLUSIONS: Shoulder and upper extremity pain was a very common problem reported by over 90% of the subjects in this study. Prevention of pain and chronic disability in athletes who use wheelchairs should be addressed by coaches, players, and health care professionals.

Adult↗

[Bilateral anterior opercular syndrome as a manifestation of a non-convulsive epileptic state].

INTRODUCTION: Foix-Chavany-Marie syndrome, or bilateral anterior opercular syndrome, is characterised by facio-pharyngo-glosso-masticatory diplegia with 'automatic-voluntary dissociation', which consists in the abolition of voluntary movements while involuntary movements and reflexes are preserved. It is produced by bilateral involvement of the anterior or frontal opercular region. In adults it is related to ischemic lesions. In childhood it presents congenitally in perisylvian dysplasias and as an acquired disorder in encephalitis or can be episodic in symptomatic or idiopathic epilepsies such as benign rolandic epilepsy. CASE REPORT: A 13-year-old patient who presented, over five straight days, four episodes of facial dysplegia, anarthria, dysphagia, drooling, paralysis of the upper limbs, while involuntary facial expression was normal and the corneal, cough and gag reflexes were preserved. The first three come to an end spontaneously at 2, 4 and 20 hours, respectively; the fourth episode concluded an hour and a half after onset, following administration of intravenous phenytoin for 5 minutes. Computerised axial tomography and magnetic resonance images of the brain, as well as the interictal electroencephalograms (EEG), were normal. Administration of oxcarbazepine was started but at 8 months was stopped after a normal EEG during nocturnal sleep was obtained. After 15 months, the patient has not presented any more episodes. CONCLUSIONS: The paroxysmal character of the disorder together with normal interictal periods, the normality of the neuroimages, and the speedy recovery achieved after the administration of phenytoin support the notion of an epileptic origin. We believe that we are dealing with a bilateral anterior opercular syndrome due to a non-convulsive epileptic state, compatible with the presentation of benign rolandic epilepsy.

Adolescent↗

Neuromuscular blockade: the effects on two hippocampal RSA (theta) systems and neocortical desynchronization.

Neocortical and hippocampal electroencephalographic (EEG) activity were recorded from rats during: (a) spontaneous activities, (b) paralysis produced by curare or gallamine neuromuscular blockade, and (c) urethane anesthesia. All of the patterns of EEG recorded from normal rats were obtained from paralyzed rats including: (a) atropine resistant neocortical desynchronization and fast (6--12 Hz) hippocampal rhythmical slow activity (RSA) normally associated with overt Type I or voluntary movement, (b) atropine sensitive and urethane resistant slow (4--6 Hz) RSA which can be recorded from immobile animals during conditioning procedures, or during brain or sensory stimulation, and (c) large amplitude irregular neocortical and hippocampal EEG which can be recorded from immobile or sleeping animals. Depth profiles of RSA were taken from the hippocampal formation of urethanized and curarized rats during posterior hypthalamic stimulation. The results confirmed findngs of two RSA amplitude maxima with an approximate 180 degree phase difference, one location in stratum oriens of area CA-1, the other in stratum moleculare of the dentate gyrus, and suggest there are two generators of RSA. Atropine sensitive and atropine resistant types of RSA were found in each generator. The results suggest that RSA may be supported by both cholinergic and noncholinergic input to each generator and that neither RSA type could be only the result of proprioceptive feedback from joints or muscles.

Animals↗

Clinical features and management of two cases of encephalitis lethargica.

Two patients with presumed encephalitis lethargica are presented with clinical features suggestive of two forms of the disease described by Von Economo: One patient had a psychosis and a mute-akinetic syndrome associated with myoclonus. The second patient presented with a psychosis and fever, developing severe dyskinesias involving the mouth, trunk and limbs, together with respiratory irregularities and presumed hypothalamic disturbance and disturbance of consciousness. In both cases, initial cerebrospinal fluid (CSF) examination revealed an elevated white cell count (predominantly lymphocytes), elevated protein in case 2, and oligoclonal bands in both cases. Computed tomography (CT) brain scan was normal but in both cases EEG revealed diffuse slow wave activity. A 18F-Dopa positron emission tomography (PET) scan in case 2 was normal. The medical management of both patients is discussed. In case 1, L-Dopa improved the akinesia, while the myoclonus responded to clonazepam. In case 2, the severe dyskinesias failed to respond to a number of drugs, and she ultimately required paralysis to relieve her almost continuous movements. Both patients responded rapidly and dramatically to intravenous methylprednisolone. We suggest that steroid treatment should be considered in the acute phase of patients with features suggestive of encephalitis lethargica.

Acyclovir↗

Dynamic reconstruction of eye closure by muscle transposition or functional muscle transplantation in facial palsy.

For patients with facial palsy, lagophthalmus is often a more serious problem than the inability to smile. Dynamic reconstruction of eye closure by muscle transposition or by free functional muscle transplantation offers a good solution for regaining near-normal eye protection without the need for implants. This is the first quantitative study of three-dimensional preoperative and postoperative lid movements in patients treated for facial paralysis. Between February of 1998 and April of 2002, 44 patients were treated for facial palsy, including reconstruction of eye closure. Temporalis muscle transposition to the eye was used in 34 cases, and a regionally differentiated part of a free gracilis muscle transplant after double cross-face nerve grafting was used in 10 cases. Patients' facial movements were documented by a three-dimensional video analysis system preoperatively and 6, 12, 18, and 24 months postoperatively. For this comparative study, only the data of patients with preoperative and 12-month postoperative measurements were included. In the 27 patients with a final result after temporalis muscle transposition for eye closure, the distance between the upper and lower eyelid points during eye closing (as for sleep) was reduced from 10.33 +/- 2.43 mm (mean +/- SD) preoperatively to 5.84 +/- 4.34 mm postoperatively on the paralyzed side, compared with 0.0 +/- 0.0 mm preoperatively and postoperatively on the contralateral healthy side. In the resting position, preoperative values for the paralyzed side changed from 15.11 +/- 1.92 mm preoperatively to 13.46 +/- 1.94 mm postoperatively, compared with 12.17 +/- 2.02 mm preoperatively and 12.05 +/- 1.95 mm postoperatively on the healthy side. In the nine patients with a final result after surgery using a part of the free gracilis muscle transplant reinnervated by a zygomatic branch of the contralateral healthy side through a cross-face nerve graft, eyelid closure changed from 10.21 +/- 2.72 mm to 1.68 +/- 1.35 mm, compared with 13.70 +/- 1.56 mm to 6.63 +/- 1.51 mm preoperatively. The average closure for the healthy side was from 11.20 +/- 3.11 mm to 0.0 +/- 0.0 mm preoperatively and from 12.70 +/- 1.95 mm to 0.0 +/- 0.0 mm postoperatively. In three cases, the resting tonus of the part of the gracilis muscle transplant around the eye had increased to an extent that muscle weakening became necessary. Temporalis muscle transposition and free functional muscle transplantation for reanimation of the eye and mouth at the same time are reliable methods for reconstructing eye closure, with clinically adequate results. Detailed analysis of the resulting facial movements led to an important improvement of the authors' operative techniques within the last few years. Thus, the number of secondary operative corrections could be significantly reduced. These qualitative and quantitative studies of the reconstructed lid movements by three-dimensional video analysis support the authors' clinical concept of temporalis muscle transposition being the first-choice method in adult patients with facial palsy. In children, free muscle transplantation is preferred for eye closure, so as not to interfere with the growth of the face by transposition of a masticatory muscle. In addition, a higher degree of central plasticity in children might be expected.

Adolescent↗

Syndrome of downward gaze paralysis, amnesia and hypersomnolence.

A 60 year old woman with computed tomographic-verified bilateral thalamic infarction is described. She demonstrated downward gaze paralysis, amnesia and hypersomnolence. On the functional level such tasks as walking and eating were disconcerting. Although there have been only a few reports of the complete triad it may be more common than realized and represents a distinct syndrome that can be identified by the bedside.

Amnesia↗

[A case of botulism in a 11-month-old infant].

We report a case of botulism in a 11 month-old infant. Hypotonia complicated by progressive bulbar paralysis revealed the disease. Botulism B toxin was present in serum on the 8th day of the disease. On the occasion of this case report the clinical, diagnostic, epidemiological and pathophysiologic aspects of infant botulism are reviewed. The relationship between sudden infant death and botulism is discussed.

Botulism↗

[Influential factors on health-related quality of life after cerebral vascular disease].

A mail survey was conducted to elucidate the influential factors on heath-related quality of life (HRQOL) after cerebral vascular disease. Questionnaires for clinicians and their patients were mailed to 2,587 hospitals with more than 100 beds, which have at least one of the following departments: neurosurgery, neurology, psychiatry or rehabilitation. Each mailing contained a request to the clinician and questionnaires for 5 cases. 378 effective questionnaires could be collected, meaning the collection rate was 2.9%. The questions for the physicians concerned diagnosis (cerebral infarction or hemorrhage), duration of illness, activities of daily living(ADL), manifestation of paralysis and psychiatric symptoms and so forth. The questionnaire for the patients was composed of items from the EuroQol clinical version (EuroQol). Geriatric Depression Scale short form (GDS) and inquiries concerning family living with the patients, their housekeeping and so on. A visual analogue scale (VAS) concerning health state of the EuroQol was used as a measure of HRQOL. Coefficients of determination between VAS and other inquiries were calculated by regression analysis or ANOVA, revealing that "anxiety/depression", "GDS" and 16 other items were statistically significant on VAS (p < 0.05). General linear model (GLM) analysis using VAS as a criterion variable and these 18 items as predictor variables showed that "sleep disturbance" and GDS score were most influential on VAS according to the F value of the type 3 sum of squares. "Health state today compared to that during the past one year", "shopping as housekeeping", "ADL" and "pain/discomfort" also have some influence on VAS. In conclusion, sleep disturbance and depression had the most deleterious effect on HRQOL.

Aged↗

Reactions to argasid tick bites by island residents on the Great Barrier Reef.

OBJECTIVE: To attribute a cause and quantify allergic-like symptoms observed among island residents. DESIGN: Skin prick tests and intradermal injections with ultraviolet-irradiated, filtered (0.22 microns) whole-body homogenates of the soft tick, Ornithodoros capensis, were used to reproduce experimentally the symptoms observed. SETTING: Heron Island, Great Barrier Reef, Australia. PARTICIPANTS: Island residents were designated as such after having spent more than 1 month on the island during the summer seabird breeding season. INTERVENTIONS: Control measures were instigated using a residual insecticide, delta-methrin, sprayed inside sleeping quarters. MAIN OUTCOME MEASURES: Acaricide spraying reduced (X2 = 4.42; P less than 0.05) the number of island residents complaining of having been bitten by ticks, yet was considered an inefficient control measure as 67% reported being attacked by ticks after spraying. RESULTS: Among 97 island residents, elevated total IgE levels were associated with reaction to tick bite in 11 cases (X2 = 27.17; P less than 0.001), but were not of reliable diagnostic value. Symptoms included intense pruritus, blistering (a major feature), erythema, weeping lesions, lymphangitis, dull ache, rheumatic pain and general lassitude, and intense discomfort. Sera from two of four volunteers with known reactions to O. capensis and one of four others with reactions to the Australian paralysis tick, Ixodes holocyclus, cross-reacted with antigens from a total of four of six biting/stinging and non-biting arthropods (radioallergosorbent tests). CONCLUSIONS: Symptoms associated with reactions to tick bite peaked in severity at 35-40 hours and thus the response was most likely delayed type IV hypersensitivity.

Animals↗

Headache in childhood.

Migraine in children is diagnosed in presence of paroxystic episodes of headache which recur with free intervals, provided intracranial diseases are excluded. Pathogenesis of this disorder is unclear; migraine with and migraine without aura may be different entities. Many factors can precipitate a migraine attack. In school age, psychologic stress is the commonest factor. Main characteristics of attacks in children are headache, which may be hemicranial; nausea and vomiting, abdominal pain, paraesthesiae. Scotomata are not very common in the pediatric age. In some cases, migraine attacks are complicated by sensory or motor symptoms (paraesthesiae, paresis), usually at one hemisoma. In the basilar artery migraine syndrome, features of brain-stem dysfunction predominate. In a few patients a migraine attack presents itself as an acute confusional state. In migraine, EEG abnormalities are frequent (predominance of diffuse or focal slowing). In some cases a CSF pleocytosis is found after an attack of complex migraine. Prognosis is good. Preventive treatment is necessary if the attacks are severe and if they cannot be relieved by rest or sleep. Symptomatic headaches may be produced by a variety of causes. Rarely, it is secondary to increased intracranial pressure. In the great majority of cases recurrent headache is due to migraine. Usually, clinical data are sufficient for diagnosis, though in some cases the diagnosis is difficult and it is necessary to perform laboratory examinations in order to exclude symptomatic headache.

Cerebrovascular Disorders↗

Bilateral vocal fold paresis and multiple system atrophy.

OBJECTIVE: To review a case series of patients with systemic neurodegenerative disease presenting to a laryngologist for workup of dysphonia and found to have bilateral vocal fold paresis. DESIGN: Case series. SETTING: Tertiary care voice center. PATIENTS: Series of patients with neurodegenerative disorders examined for dysphonia. MAIN OUTCOME MEASURES: History and physical examination including fiberoptic laryngoscopy were performed on all patients. Some patients underwent polysomnography. RESULTS: Seven patients during a 2-year period were noted to have bilateral abductor vocal fold paresis. Five of 7 (71%) had the diagnosis of multiple system atrophy proposed by the laryngologist. All 7 patients described sleep-disordered breathing with stridor. CONCLUSIONS: Patients with systemic neurodegenerative disorders such as Parkinson disease should be examined for multiple system atrophy and for evidence of bilateral vocal fold paresis. Workup for stridor should include polysomnography. Treatment of glottic obstruction in these patients includes constant positive airway pressure at night or tracheotomy. The finding of bilateral vocal fold paresis can be life threatening.

Aged↗