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Opisthotonus and other unusual neurological sequelae after outpatient anaesthesia.

Four patients who developed unusual neurological sequelae after outpatient anaesthesia are described. Propofol is strongly implicated as the cause. All four patients were female with no previous history of psychiatric disorder or neurological disease, unpremedicated, and had procedures of duration less than 20 minutes. Hyperreflexia and hypertonicity were present postoperatively and the reactions appeared to be triggered by an external stimulus. Three patients were examined by a neurologist and had a normal electroencephalograph. Two patients were on the same operating list; quality control was carried out on the anaesthetic agents used, and blood samples sent for toxicology showed no abnormalities. Mechanisms underlying these reactions are discussed.

Adult↗

Electrophysiologic studies in Miller Fisher syndrome.

Serial peripheral nerve conduction studies are reported on a case of acute ophthalmoplegia and ataxia with areflexia (Miller Fisher syndrome). These tests showed abnormalities identical to those found in the Guillain-Barré syndrome.

Ataxia↗

Cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS): a new syndrome.

There are a large number of well recognised syndromes comprising cerebellar ataxia in association with other neurological features. We report three family members who presented with a relapsing, early onset cerebellar ataxia, associated with progressive optic atrophy and sensorineural deafness. All three patients have areflexia (in the absence of a peripheral neuropathy), a pes cavus deformity, and show varying degrees of severity. Extensive neurological investigations have been normal, and the aetiology and pathophysiology of this disorder remain unclear. This may represent a separate syndrome of early onset cerebellar ataxia with associated features ("cerebellar ataxia plus"), which is likely to either have an autosomal dominant or maternal mitochondrial pattern of inheritance. The recognition of this association under the acronym of CAPOS (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural deafness) may help in the delineation of a new syndrome.

Abnormalities, Multiple↗

Motor profile of patients in minimally responsive and persistent vegetative states.

The purpose of this descriptive clinical study is to document the motor characteristics of patients in minimally responsive and persistent vegetative states. Twelve subjects, presenting a prolonged altered state of consciousness (x = 7.82 years, range 2-27), aged 27-78 years (x = 50, SD = 15.26) were evaluated, using standardized protocols, on the following variables: passive range of motion, observed movements, reflexes, tonus, postural status and reactions. The subjects' level of awareness and responsivity were measured with the Coma/Near Coma (CNC) scale at each of the three data collection sessions. While group CNC scores were stable over the three sessions, fluctuations in the level of awareness of individual subjects was recorded, confirming the heterogeneity of this clientele. Abnormal primitive reflexes were present in all subjects, with the flexor withdrawal (75%), the tonic labyrinthine (36%) and the body-on-body righting reaction (25%) being the most frequently observed. All subjects presented altered tonus, considerable posturing and varied degrees of reduced range of joint motion. A range of abnormal (e.g. chewing, clonus) and normal patterned (e.g. bridging, scratching) movement behaviours was recorded, but these did not translate into functional use, such as rolling. Collectively, the findings stress the complexity of the motor profile of patients in minimally responsive and vegetative states, and suggest the need for physiotherapists to become more actively involved in the evaluation and treatment of this clientele.

Adult↗

Observations on the Tullio phenomenon.

Vestibular responses (vertigo, nystagmus-like eye movements) to acoustic stimuli are known as the "Tullio phenomenon". Detailed electro-oculographic analysis of this reaction, as observed in a 30-year-old patient, revealed the following: a maximum amplitude of eye movement (mainly vertical) was achieved by sine wave bursts of high intensity, a frequency of 500 to 1000 Hz and a duration of 100 ms. The ocular deviation was composed of a fast initial component, followed by a slower resetting movement that was often divided into two parts of different velocities. At longer stimulus durations (more than 100 ms) the electro-oculogram showed a fractionation of the eye deviation, terminating in an "off-response". Various positions of the patient's head influenced the direction of the eye motion. The possibility that the Tullio phenomenon may be due to an abnormal excitation of the statolith organs is discussed.

Acoustic Maculae↗

A prospective study to identify high-yield criteria associated with acute intracranial computed tomography findings in head-injured patients.

We conducted a multicenter, prospective study of head-injured patients to identify high-yield clinical criteria for acute intracranial injuries. Emergency patients with a history of blunt head trauma occurring within 2 weeks and who underwent nonenhanced cranial computed tomography (CT) were entered onto the study during a 12-month period. Of the 264 patients, 32 (12%) had abnormal CT findings. Nine high-yield variables were associated with abnormal CT findings: alcohol use before injury, antegrade amnesia, prolonged loss of consciousness, anisocoria and/or fixed and dilated pupils, abnormal Babinski reflex, focal motor paralysis, cranial nerve deficit, Glasgow coma scale score of less than 15, and clinical signs of basilar skull fracture. Patients 2 years old or younger or older than 60 years of age showed a significantly greater prevalence of abnormal CT findings than patients of other ages.

Acute Disease↗

Normal pressure hydrocephalus. Recognition and relationship to neurological abnormalities in Cockayne's syndrome.

Normal pressure hydrocephalus (NPH) in adults is a well-known cause of dementia. We describe NPH in children having the recessively inherited Cockayne's syndrome (CS). Cockayne's syndrome is characterized by cachectic dwarfism, neurological dysfunction, and cutaneous sunlight sensitivity. We noted that the NPH-associated triad of dementia, gait disturbance, and incontinence developed in CS patients. Computerized tomography of the brain in our four CS patients showed hydrocephalic enlargement of the brain ventricles greatest in the older patients. There was no evidence of cortical atrophy except in the one patient who had CS with xeroderma pigmentosum. Lumbar puncture and radionuclide cisternography in the two patients tested showed normal CSF pressure, with complete blockade to flow of radionuclide above the tentorium cerebelli, ventricular reflux, and delayed absorption. Studies of NPH in CS may elucidate the pathophysiology of NPH and methods to alter its sequelae.

Abnormalities, Multiple↗

A review of the Amiel-Tison neurologic evaluation of the newborn and infant.

Because occupational therapists are becoming more involved in infant assessment and intervention, there is a need for objective, prospective, and serially applicable evaluation tools. Amiel-Tison's Neurologic Evaluation of the Newborn and the Infant provides such a tool for use in the first year of life. This evaluation was developed to detect transient and permanent abnormalities in an infant's neuromotor development. Its main focus is to examine active and passive muscle tone. The test has interobserver reliability, is reproducible, and can be readily learned by occupational therapists. Although the evaluation is not standardized, it is clinically useful in neonatal units, developmental clinics, and research settings. This evaluation detects transient neuromotor problems in the first year of life that are associated with significant behavioral, neurological, and intellectual deficits when the children reach school age. Using this test, occupational therapists frequently involved with the assessment and management of children can play a crucial role in the first year of life by diagnosing and documenting abnormalities.

Humans↗

Aspiration in bilateral stroke patients: a validation study.

We validated the predictive accuracy of an "old" regression model in a "new" sample of bilateral stroke patients (N = 38). Abnormal gag reflex and impaired voluntary cough accurately predicted radiographically verified aspiration in both samples. A final model, using both samples, grouped patients into three risk strata: low risk of 14% (cough and gag normal), moderate risk of 46% to 51% (one of two behaviors abnormal); and high risk of 87% (cough and gag abnormal).

Adult↗

Neurological manifestations of accidental hypothermia.

In a series of 97 patients with accidental hypothermia, alcohol abuse and Wernicke's encephalopathy were prominent causes. Pulse, systolic blood pressure, and respiratory rate were all found to decline with decreasing temperature, and there were significant changes (p less than 0.01) in level of consciousness, pupillary response, reflexes, and muscle tone. However, even in the lowest temperature range (20 degrees to 27 degrees C, or 68 degrees to 80 degrees F), 6 of 18 patients remained verbally responsive and 10 had intact reflexes. Neither eye movement abnormalities nor extensor plantar responses correlated directly with the degree of hypothermia.

Blood Pressure↗

Autonomic and sensory nerve dysfunction in primary biliary cirrhosis.

AIM: Cardiovascular autonomic and peripheral sensory neuropathy is a known complication of chronic alcoholic and non-alcoholic liver diseases. We aimed to assess the prevalence and risk factors for peripheral sensory nerve and autonomic dysfunction using sensitive methods in patients with primary biliary cirrhosis (PBC). METHODS: Twenty-four AMA M2 positive female patients with clinical, biochemical and histological evidence of PBC and 20 age matched healthy female subjects were studied. Five standard cardiovascular reflex tests and 24-h heart rate variability (HRV) analysis were performed to define autonomic function. Peripheral sensory nerve function on median and peroneal nerves was characterized by current perception threshold (CPT), measured by a neuroselective diagnostic stimulator (Neurotron, Baltimore, MD). RESULTS: Fourteen of 24 patients (58%) had at least one abnormal cardiovascular reflex test and thirteen (54%) had peripheral sensory neuropathy. Lower heart rate response to deep breathing (P = 0.001), standing (P = 0.03) and Valsalva manoeuvre (P = 0.01), and more profound decrease of blood pressure after standing (P = 0.03) was found in PBC patients than in controls. As a novel finding we proved that both time domain and frequency domain parameters of 24-h HRV were significantly reduced in PBC patients compared to controls. Each patient had at least one abnormal parameter of HRV. Lower CPT values indicated hyperaesthesia as a characteristic feature at peroneal nerve testing at three frequencies (2000 Hz: P = 0.005; 250 Hz: P = 0.002; 5 Hz: P = 0.004) in PBC compared to controls. Correlation of autonomic dysfunction with the severity and duration of the disease was observed. Lower total power of HRV correlated with lower CPT values at median nerve testing at 250 Hz (P = 0.0001) and at 5 Hz (P = 0.002), as well as with those at peroneal nerve testing at 2000 Hz (P = 0.01). CONCLUSION: Autonomic and sensory nerve dysfunctions are frequent in PBC. Twenty-four-hour HRV analysis is more sensitive than standard cardiovascular tests for detecting of both parasympathetic and sympathetic impairments. Our novel data suggest that hyperaesthesia is a characteristic feature of peripheral sensory neuropathy and might contribute to itching in PBC. Autonomic dysfunction is related to the duration and severity of PBC.

Aged↗

[Cortical reflex myoclonus in adult onset Huntington's disease].

We report a case of cortical reflex myoclonus in adult onset Huntington's disease (HD). The patient is a 51-year-old woman. Chorea and myoclonus were observed on her face and extremities. Neurophysiological tests showed C reflex and abnormal waves preceding myoclonus by jerk-locked back averaging method but no giant somatosensory evoked potential. Gene analysis revealed the prolongation of CAG repeats (13/44) in IT15 gene. Oral administration of clonazepam was transiently effective for myoclonus. We should inscribe that the cortical reflex myoclonus may exceptionally manifest in HD.

Cerebral Cortex↗

Poliomyelitis-like syndrome associated with asthma. A case report and review of the literature.

A 5-year-old asthmatic boy had flaccid monoplegia six days after an asthma attack. A similar association has been reported in 13 previous cases. However, to our knowledge, this is the first report from the American continent. This syndrome has a poor prognosis and can be confused with poliomyelitis. The localizing effect of abnormal inspiratory-biceps synkinesis may be important.

Asthma↗

Testicular pain sensation in diabetic autonomic neuropathy.

Testicular sensation was examined in 32 male diabetics with symptoms suggestive of autonomic neuropathy to evaluate its usefulness as a clinical sign in this condition. Vascular reflex responses to the Valsalva manoeuvre and sustained handgrip and blood pressure fall on standing were measured as objective tests of autonomic involvement. Absent or diminished testicular sensation correlated well both with the clinical features of autonomic neuropathy, except where impotence occurred alone, and with abnormal vascular reflexes. Most patients with impotence alone had normal testicular sensation and normal vascular reflexes. It is concluded that this sign is useful in diabetics with impotence, the presence of intact testicular sensation indicating that the impotence is unlikely to be due to autonomic neuropathy.

Achilles Tendon↗