Third cranial nerve palsy or pseudo 3rd nerve palsy of myasthenia gravis? A challenging diagnosis in systemic lupus erythematosus.

Diplopia is frequently encountered in neurological practice and may occur as part of the clinical spectrum of 3rd cranial nerve palsy. Correct localization of the underlying problem is the first step in making an accurate diagnosis. Pathologies affecting the supranuclear structures, cranial nerves and nuclei, extraocular muscles and the neuromuscular junction may cause or simulate 3rd nerve palsy. We report a case of a patient with longstanding quiescent SLE who presented with sudden onset of diplopia and discuss possible aetiologies including ischemia, demyelination, neuromuscular conditions and medication-induced.

The relation between initial symptoms and signs and the prognosis of whiplash.

Whiplash, a common injury following motor vehicle crashes, is associated with high costs and a prognosis that is variable and difficult to predict. We studied the profile of recovery from whiplash and assessed whether presenting signs and symptoms directly after the crash were predictive of whiplash prognosis. We formed a population-based incident cohort of all 2627 individuals who sustained a whiplash injury resulting from a motor vehicle crash in the province of Québec, Canada, in 1987, and followed these patients for up to 7 years. The data on signs and symptoms were obtained from the medical charts kept by the universal automobile insurance plan (Société de l'assurance automobile du Québec), which covers all 7 million residents of the province, while data on the outcome--the recovery time from whiplash--was obtained from their databases. The median recovery time was 32 days, and 12% of subjects had still not recovered after 6 months. The signs and symptoms that were found to be independently associated with a slower recovery from whiplash, besides female gender and older age, are neck pain on palpation, muscle pain, pain or numbness radiating from the neck to arms, hands or shoulders, and headache. Together, these factors in older females (age 60) predicted a median recovery time of 262 days, compared with 17 days for younger males (age 20) who do not have this profile. In contrast, using a classification of injury severity previously proposed by the Québec Whiplash Associated Disorders Task Force, the median recovery time varied from 17 to only 123 days. We conclude that whiplash patients presenting with several specific musculoskeletal and neurological signs and symptoms will have a longer recovery period. These patients can easily be identified and closely monitored and targeted for the evaluation of early intervention programmes aimed at managing whiplash patients with a poor prognosis.

Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin.

Acute disseminated encephalomyelitis (ADEM) is a presumed immune-mediated, demyelinating disease of the CNS for which the standard treatment is high-dose steroids. We describe two patients with ADEM in whom treatment with IV methylprednisolone coincided with deterioration in their clinical status. They were subsequently treated with IV immunoglobulin and exhibited dramatic clinical improvement, with return to their previous level of functioning.

Proximal median neuropathy secondary to humeral neck fracture.

Median neuropathies proximal to the wrist are uncommon and usually result from penetrating injuries, fracture dislocation of the distal humerus, or compression by fibrous bands. A 66-year-old man suffered a comminuted fracture of the proximal humerus after a fall. Electrodiagnostic studies revealed a severe proximal median neuropathy and a mild distal radial mononeuropathy. Proximal median neuropathy rarely occurs in humeral neck fracture, mostly because the median nerve is not in close contact with the humerus proximally.

Use of evidence in the process of practice change in a clinical team: a study-forming part of the Autocontrol Project.

The Autocontrol Project is concerned with the accessing, processing and communication of high quality information so that a clinical team can make and implement decisions for practice change, and then evaluate if improvement has been achieved. High quality information is used as evidence for change. In this study, we have evaluated how evidence is used by a clinical team to explain an identified problem of inappropriate use of blood gas tests. In an experimental study of the Surgical Intensive Care Unit, video recordings of team meetings of nurses and doctors were undertaken, structured according to a problem-based format. Evidence of current practice patterns derived from the hospital information system, as well as the results of a questionnaire to the unit's staff about knowledge and use of blood gas measurements, were supplied to the participants beforehand. At the second meeting, the output of the first meetings and a summarised analysis of pertinent literature were made available. This second meeting was required to finalise the list of causes of inappropriate blood gas use and propose pragmatic strategies for practice change. The video data of the meetings were coded to analyse the use of evidence, the categories of causes, issues and solutions proposed, and the quality of team interaction. The results indicate that in order to achieve consensus, the team used different types of evidence, including objective evidence of practice patterns, personal experience about direct and indirect organisational influences, and literature-based research evidence of best practice. Furthermore, group dynamics were favoured by the problem-based meeting structure, and a high level of cognitive critiquing between team members was observed. This research suggests that a combination of approaches involving identification of both operational factors (e.g. appropriate access to different types of evidence and meeting structure) and cognitive and behavioural approaches (e.g. ensuring expression of different viewpoints) is needed to support strategic decision-making for practice change in a clinical unit. This combined approach should favourably influence the provision of an effective and efficient evidence support environment for the clinical team.

The effect of socio-demographic and crash-related factors on the prognosis of whiplash.

Whiplash injury, common after a motor vehicle crash, has a variable prognosis that is difficult to predict. To assess the role of various factors on this prognosis, we assembled a historical cohort of 3014 individuals who sustained a whiplash injury resulting from a motor vehicle crash in the Province of Quebec, Canada, in 1987 and were followed for 6 years. The data were obtained from the computerized databases created by the province's universal automobile insurance plan and police accident reports. The recovery time from whiplash, as measured by duration of compensation, was the primary outcome. Socio-demographic and crash-related factors measured at the time of the crash were investigated. The median recovery time for the cohort was 31 days, with 22% recovering within a week and 3% still not recovered after 1 year. For the 1551 subjects with a whiplash injury only, the socio-demographic factors that were found to be independently associated with a slower recovery from whiplash in this cohort are female gender, older age, having dependents, and not having full-time employment. The significant crash-related factors are occupancy in a truck or bus, being a passenger in the vehicle, colliding with a moving object, and being in a head-on or perpendicular collision. We classified the subjects according to a prediction score ranging from 0 to 11, devised from these factors. Subjects with a score of 0 to 2, that is those who had at most two risk factors present, had the fastest median recovery time of 19 days compared with 71 days for subjects who had a score of 6 or more. We conclude that several sociodemographic and crash-related factors are independently associated with a slow and costly recovery from whiplash injury. They are easily measurable at the time of the crash and combined so as to be simply incorporated in intervention programs aimed at early identification and management of whiplash patients with a poor prognosis.

Clinical correlates of subnormal vitamin B12 levels in patients infected with the human immunodeficiency virus.

OBJECTIVES: To determine the prevalence and describe the clinical correlates of subnormal cobalamin levels in subjects infected with the human immunodeficiency virus (HIV), and to assess its relationship to virus-mediated immunosuppression and/or anti-viral therapy. SETTING: Outpatient referral clinic in tertiary care hospital. PATIENT POPULATION: 200 HIV infected individuals. STUDY DESIGN: Descriptive cross sectional survey, with prospective follow-up in a subgroup of patients before and after initiation of zidovudine therapy. MEASURES: Routine complete blood count, serum B12 assay, CD4 counts. Serum homocysteine levels, and Schilling tests were performed on subgroups of study subjects. RESULTS: Subnormal serum B12 levels were found in 61 subjects (30.5%). B12 deficient subjects were more likely to be taking zidovudine. (P = .007). Serum homocysteine levels were significantly higher in patients with subnormal cobalamin levels but were unrelated to CD4 counts or zidovudine use, and were rarely outside of the normal range. Malabsorption of vitamin B12 as evidenced by abnormal Schilling tests was more likely among patients with more advanced HIV disease, or gastrointestinal symptoms but was not necessarily associated with low B12 levels. CONCLUSIONS: Decreased cobalamin levels are found frequently in HIV disease, especially among those treated with zidovudine. Evidence of B12 malabsorption is found among those with more advanced disease and gastrointestinal symptoms.

Sensorimotor neuropathy and abnormal vitamin B12 metabolism in early HIV infection.

Distal sensory peripheral neuropathy (DSPN) has been reported in 5 to 75% of patients with human immunodeficiency virus (HIV) infection, particularly in advanced stages of the disease. Twenty HIV seropositive patients were studied prospectively to determine the frequency of DSPN in clinical stage II and III of the HIV infection, and to investigate the role of vitamin B12 deficiency on the frequency of DSPN in HIV patients. All patients had complete blood count, serum vitamin B12 level, anti-intrinsic factor antibody, Schilling test, and electrodiagnostic studies including nerve conduction studies and concentric needle examination in the lower extremities, and sympathetic skin responses. Only 1 patient (5%) had clinical and electrophysiological evidence of possible DSPN. Of the 6 patients with abnormal Schilling test, only one had DSPN based on distal sensory symptoms, abnormal neurological examination and electrodiagnostic studies. Evidence for possible DSPN was present in 5% of patients with early HIV infection and did not appear to be more frequent in patients with concurrent vitamin B12 deficiency.

Prognostic implications of transient left ventricular cavitary dilation during exercise and dipyridamole-thallium imaging.

OBJECTIVE: To determine the prognostic implication of exercise and dipyridamole-induced transient left ventricular cavitary dilation (TLVD). DESIGN: TLVD was observed and a follow-up obtained in 61 patients after exercise and in 62 patients following dipyridamole infusion. PATIENTS: There was no statistical difference between groups in terms of sex, history of hypertension, diabetes, renal failure, previous myocardial infarction, severity of angina syndrome, congestive heart failure, resting electrocardiographic (ECG) abnormalities, clinical or ECG signs of ischemia during stress, number of reversible perfusion defects on thallium images or duration of follow-up (21 months). RESULTS: Dipyridamole patients were slightly older (64 versus 57 years) and displayed more thallium redistribution (P = 0.002). After a mean follow-up of 21 months, both fatal and nonfatal (myocardial infarction or cardiac death) cardiac events were more frequent in the dipyridamole group (50% versus 9%, P = 0.0001). CONCLUSIONS: Patients with dipyridamole-induced TLVD are at greater risk than those with exercise-induced TLVD at the authors' institution.

Anterior interosseous neuropathy in the postpartum period.

The anterior interosseous neuropathy is a rare focal neuropathy with typical clinical and electromyographic features. Most commonly reported etiologies include lesion of the median nerve following fracture of the radius and ulna, acute or repeated trauma or prolonged pressure on the forearm. In some cases, no predisposing factors can be elicited. Over a one-year period, two young women in their late twenties were evaluated for weakness of the flexor pollicis longus, flexor digitorum profundus of the 2nd and 3rd fingers, and pronator quadratus muscles that occurred within a month following parturition. Nerve conduction studies and concentric needle electrode examination of the upper extremities performed respectively 3 and 10 months after the onset of symptoms confirmed a severe anterior interosseous neuropathy and excluded more common conditions such as carpal tunnel syndrome, cervical radiculopathy or brachial plexopathy. The prognosis was unfavorable in both cases.

Paraneoplastic encephalomyelitis and subacute dysautonomia due to an occult atypical carcinoid tumour of the lung.

A case of paraneoplastic encephalomyelitis and subacute pandysautonomia associated with an occult atypical carcinoid tumour of the lung is described. The main clinical features were lethargy, impaired memory, constipation, and orthostatic hypotension. Neurological investigation was unremarkable except for mononuclear pleocytosis and increased protein level in the cerebrospinal fluid (CSF). Tests of autonomic function revealed a low plasma norepinephrine level, a marked drop of blood pressure (BP) to vertical tilt and Valsalva maneuver, and a marked rise of BP to dilute norepinephrine infusion. A few days prior to death, the patient became hypothermic and had repeated episodes of respiratory arrest associated with transient atrioventricular block on the electrocardiogram (ECG). A polysomnographic study confirmed a sleep apnea syndrome. Autopsy revealed an atypical carcinoid tumour in one tracheobronchial lymph node, widespread lymphocytic infiltrates and loss of neurons in the cerebral, cerebellar and brainstem grey matter, the spinal cord and roots, and the paravertebral sympathetic ganglia as well as microglial and astrocytic proliferation in the central nervous system.

Restless legs syndrome and nocturnal myoclonus: initial clinical manifestation of familial amyloid polyneuropathy.

Restless legs syndrome was the first isolated clinical manifestation in four siblings of a family with familial amyloid polyneuropathy. Clinical and electrophysiological evidence of peripheral neuropathy appeared after a variable time interval. Polysomnography showed abnormal sleep patterns and nocturnal myoclonus in all patients. The restless legs syndrome responded favourably to clonazepam.

Somatosensory evoked potentials: lack of value for diagnosis of thoracic outlet syndrome.

Twenty patients with thoracic outlet syndrome (TOS) seen at the Mayo Clinic between October 1984 and November 1985 were studied prospectively with routine nerve conduction studies, concentric needle examination, and bilateral median and ulnar somatosensory evoked potentials (SEPs). Results of nerve conduction studies and needle examination were abnormal in 30% of the patients, one patient having a reduced ulnar sensory nerve action potential amplitude and five others having neurogenic motor unit potential changes in the hand muscles. Ulnar SEPs were abnormal in three patients (15%), and median SEPs were abnormal in one patient, who also had abnormalities in ulnar SEPs. In patients with TOS, routine nerve conduction studies and needle examination were the most helpful electrophysiologic studies in excluding more common conditions. The routine use of ulnar SEPs in the evaluation of patients with TOS is probably not worthwhile.

The value of ulnar somatosensory evoked potentials (SEPs) in cervical myelopathy.

In 57 patients with clinical signs and surgical documentation of compressive myelopathy, ulnar nerve somatosensory evoked potentials (SEPs) were more sensitive (with 74% abnormal) than either median or tibial nerve SEPs. The most frequent abnormalities were reduced or absent neck evoked responses and prolonged central conduction time. All subjects who had an SEP abnormality were identified by combined tibial and ulnar SEPs. Median nerve SEP added no additional information. Normal ulnar and tibial nerve SEPs were also able to exclude major cord damage in patients with cervical radiculopathy but little evidence of myelopathy.

Syringomyelia: electrophysiologic aspects.

The clinical and EMG findings in 44 patients with syringomyelia who were seen at the Mayo Clinic between 1976 and 1985 are presented. In 10 of the patients, somatosensory evoked potentials (SEPs) of the upper and lower extremities were obtained. All 44 patients had radiographic or surgical evidence of a cervical syrinx. The most common abnormality on nerve conduction studies was a reduced hypothenar compound muscle action potential amplitude (23 patients). Abnormal findings on needle electromyography were present in 33 patients and included sparse fibrillation potentials, reduced motor unit potential (MUP) recruitment, and chronic neurogenic MUP changes in muscles innervated by the C-5, T-1 roots, with the most pronounced changes in small hand muscles. Ulnar and median nerve SEPs were usually normal in the presence of a dissociated sensory loss and were usually abnormal when all sensory modalities were impaired. Abnormalities of tibial nerve SEPs were frequent and were related to impaired proprioceptive sensation in the lower extremities.