Predicting mortality in intensive care unit patients with stroke.
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APACHE↗
Biomedical subjects
J P Stübgen
Publications and source records attributed to J P Stübgen.
Unilateral Adie pupil as sole ophthalmic sign of anti-Hu paraneoplastic syndrome.
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Aged↗
Synovial fluid polymerase chain reaction as an aid to the diagnosis of central nervous system Whipple's disease.
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Aged↗
Nervous system lupus mimics limbic encephalitis.
A 28-year-old woman with systemic lupus erythematosus (SLE) suffered recent onset fever, headache, encephalopathy followed by severe, repeated generalized seizures. Investigations revealed limbic encephalitis. Tests for Herpes simplex encephalitis and paraneoplastic encephalomyelitis were negative. High titers anti-ribosomal-P antibodies in the cerebrospinal fluid (CSF) suggested an association with nervous system lupus. No brain biopsy was performed. Treatment was with anti-seizure, anti-viral, and immunomodulating medication.
Adult↗
Limb girdle muscular dystrophy: a prospective follow-up study of functional impairment.
This 6-year prospective follow-up study evaluated the rate of clinical disease progression in 19 of 20 previously reported patients with strictly selected limb girdle muscular dystrophy. There was no significant deterioration in muscle strength (assessed by manual muscle testing). An activities of daily living (ADL) scale showed significant functional deterioration in 30% of patients with predominant involvement in a single functional domain variable between patients, and determined by the patient's previous level of function. A functional grading system showed that 70% of patients had deteriorated at least one grade in the arms and/or legs. Deterioration of the ADL score correlated with the loss of functional grades. We conclude that assessment of functional ability may be more sensitive to detect clinical disease progression compared to testing of muscle strength. The clinical parameter that correlates best with the activity of the underlying disease process is not determined. Tests for strength and functional impairment are complementary. Patients expressed appreciation that functional disability was addressed during follow-up visits.
Activities of Daily Living↗
Neuromuscular disorders in systemic malignancy.
Paraneoplastic neuronopathies are presumed to be the result of an autoimmune attack directed at neuronal proteins, and both humoral and cell-mediated mechanisms have been postulated. The lower motor neuron syndrome after irradiation to the spinal column is caused by a proximal motor polyradiculopathy. Prevention of brachial plexopathy after radiotherapy for breast cancer may be accomplished by lower doses and surgical management of the axilla. Polymerase chain reaction casts doubt on the distinction between neoplastic and paraneoplastic mechanisms of neuromuscular manifestations of lymphoproliferative diseases. Shared antigenic components may underlie the association between inflammatory neuropathy and malignant melanoma. Advances in chemotherapy strategies against responsive tumors are hindered by the toxic effects of agents on peripheral nerves.
Animals↗
Limb girdle muscular dystrophy: a radiologic and manometric study of the pharynx and esophagus.
Limb girdle muscular dystrophy (LGMD) is not a recognized cause of dysphagia. However, a systematic study of pharyngoesophageal function in LGMD has not been performed or reported. We determined whether the dystrophic process involves the pharyngoesophageal musculature in 20 LGMD patients with and without complaints of deglutition. Pharyngeal and esophageal function was evaluated by conventional cineradiography and manometry. Abnormalities were demonstrated in 30% (6/20) of patients: dysphagia in 10% (2/20), an abnormal radiologic study in 30% (6/20), and an abnormal manometric study in 20% (4/20). Mean manometric pressures were not significantly different when patients were compared with a healthy, age- and sex-matched volunteer group. In 2 patients, dysfunction of the pharyngeal striated muscle was likely, or possibly, due to dystrophic affection of the upper alimentary tract. Significant upper alimentary tract dysfunction in LGMD is not common. The cause-effect relationship between the dystrophic process and the nonspecific pharyngoesophageal motility disorders is unclear and requires pathologic study.
Adult↗
Palliative care rounds: T12 epidural metastasis mimicks pelvic recurrence of cecal adenocarcinoma.
Following resection of an adenocarcinoma of the cecum, a 59-yr-old patient developed pelvic pain. Imaging of the pelvis was unrevealing, but magnetic resonance imaging of the spine demonstrated a T12 epidural lesion. Pelvic pain may be due to an upper lumbar or lower thoracic radiculopathy. This referral pattern must be recognized when evaluating cancer patients with occult pelvic pain.
Adenocarcinoma↗
Neuromuscular disorders in systemic malignancy and its treatment.
Neuromuscular dysfunction in patients with known or suspected malignancy has three basic etiologies: (1) a direct effect of the neoplasm, either by compression or infiltration; (2) a "remote," or paraneoplastic, effect of cancer; or (3) a side effect of anticancer treatment, radiation or chemotherapy. A variety of clinical features or syndromes are due to damage either at the level of the neuron (anterior horn cell or dorsal root ganglion neuron), nerve root(s), brachial or lumbosacral plexus, peripheral nerve (motor, sensory, and/or autonomic), neuromuscular junction, or muscle. A complex clinical picture evolves when dysfunction in due to more than one cause at more than one anatomical site.
Antineoplastic Agents↗
Intraventricular blood after "traumatic" lumbar puncture: a report of two cases.
Blood was detected in the lateral ventricles on head computed tomograms performed after "traumatic" lumbar punctures in two children. This finding has not previously been observed or reported. The likely mechanisms by which blood from the spinal thecal sac reached the ventricles are discussed. This potentially confusing observation, if not correctly accounted for, may lead to an unnecessary, fruitless search for the site of a supposed "primary" intraventricular haemorrhage.
Cerebral Hemorrhage↗
Neurosarcoidosis presenting as a retroclival mass.
A head magnetic resonance imaging scan on a 22-year-old black woman with a 1-year history of brain stem symptoms showed a retroclival mass. Noninvasive studies were unhelpful in characterizing the lesion. Biopsy of the mass showed noncaseating granulomas, consistant with sarcoidosis. Symptoms improved on steroid treatment. Sarcoidosis should be considered in the differential diagnosis of a retroclival mass; difficulties in the noninvasive investigation of neurosarcoidosis are discussed.
Adult↗
Crossed cerebellar diaschisis related to recurrent focal seizures.
After several days of recurrent focal motor seizures in a 32-year-old pregnant woman, a brain magnetic resonance imaging (MRI) scan showed signal abnormalities restricted to cerebral cortex and contralateral cerebellum that did not enhance after gadolinium administration. Maximal EEG dysfunction and seizure onset correlated anatomically with the area of image change. An aggressive medication regimen and termination of the pregnancy resulted in seizure control, reversal of postictal neurologic deficit, and improvement or resolution of the MRI and EEG abnormalities in 6 weeks. We concluded that the sequence of events suggested the reversible MRI lesions to be the result of repetitive seizure activity and that both localization and resolution of the lesions may be explained by reversible excitotoxic cell damage due to seizure-related excessive synaptic discharge.
Adult↗
Limb girdle muscular dystrophy: a quantitative electromyographic study.
BACKGROUND: Quantitative electromyography (EMG) using different needle techniques has not been performed on a large limb girdle muscular dystrophy (LGMD) group. PURPOSE: To establish statistically: (1) correlations between clinical features (patient age, disease duration, degree of weakness) and quantitative needle EMG; (2) correlations between different needle EMG parameters, and (3) differences in quantitative EMG comparing patients with a healthy control group. METHODS: Nerve conduction studies and needle EMG (motorunit analysis, MacroEMG, SFEMG) were performed on Mm. triceps brachii and tibialis anterior according to standard techniques on 20 LGMD patients. RESULTS: Nerve conduction studies were essentially normal. Motorunit analysis and MacroEMG parameters correlated with various clinical features. SFEMG results did not correlate with either clinical measures or other needle EMG findings. There was a significant difference between patients and controls in motorunit analysis and MacroEMG results. CONCLUSION: (1) Progressive weakness correlated with concentric needle and MacroEMG indicators of progressive muscle fibre degeneration and loss. (2) SFEMG showed that muscle fibre reinnervation followed a course independent of fibre degeneration, and measured clinical features.
Action Potentials↗
Limb girdle muscular dystrophy: weakness and disease duration as predictors of functional impairment.
This cross-sectional study compared progressive weakness to functional disability in 20 patients with limb girdle muscular dystrophy. Weakness (assessed by manual muscle testing) determined disability (assessed by functional grades, timed functional tests, and an activities of daily living scale). For any functional grade the degree of weakness varied between patients. Yet, at a certain "threshold" weakness the functional level predictably deteriorated. Change in functional grade did not reflect a parallel increase in weakness nor disease progression. Functional "milestones" were reached at varying durations of disease. Only guidelines were offered to individual patients as to the time course of progression in disability. Serial timed tests should be a sensitive, objective method to follow the rate of disease progression. Timely referral of patients to establish physical needs should delay functional deterioration and minimize handicap despite progressive weakness.
Activities of Daily Living↗
Limb girdle muscular dystrophy: description of a phenotype.
The phenotype is reported of 20 patients with autosomal recessive or sporadic, pelvifemoral limb girdle muscular dystrophy (LGMD). Selective wasting of muscles was observed at the moderately advanced stage of illness. The pattern of weakness was uniform. Attention to clinical detail allowed the identification of a phenotype different from a hypothetical scheme of LGMD based on previous literature, and other causes of limb girdle weakness. These patients may represent yet another nosologic entity within the autosomal recessive dystrophies; molecular genetic studies are awaited. A limited magnetic resonance imaging (MRI) study of muscle was of little consequence. Although additional detail was obtained, no pathognomonic distribution of the dystrophic process was observed; interindividual variation existed even among closely matched siblings. The severity of MRI signal change did not consistently correlate with the degree of weakness in an individual. When a diagnosis is uncertain, however, the added detail may be useful.
Adult↗
Lung and respiratory muscle function in limb girdle muscular dystrophy.
BACKGROUND: Pulmonary involvement is frequently observed in patients with limb girdle muscular dystrophy and occurs early in the disease. The aim of this study was to establish the prevalence of pulmonary dysfunction; the type of dysfunction; and any correlation between patient age, disease duration, or limb weakness and lung or respiratory muscle dysfunction. METHODS: Twenty patients with strictly delineated limb girdle muscular dystrophy and 20 healthy controls were evaluated. Full inspiration chest radiographs were obtained. Standard lung and respiratory muscle function tests were performed and the data were statistically analysed. RESULTS: The mean age of the patients was 40.6 years, the mean disease duration was 18.9 years, and the mean average muscle score (a numerical expression of limb weakness) was 5.73 out of 10. Chest radiography showed unilateral paresis of the diaphragm in three patients. Increased residual volumes, with either increased or decreased total lung capacity, correlated inversely with disease duration. Respiratory muscle weakness was common but mild. Expiratory muscle function was more impaired than inspiratory muscle function and correlated positively with expiratory reserve volume. CONCLUSIONS: Respiratory muscle strength is commonly impaired in limb girdle muscle dystrophy. A dissociation of the limb and mild respiratory muscle involvement is observed; wheelchair restriction does not predict worsening of pulmonary function, and patient age, disease duration, or degree of limb weakness do not predict pulmonary morbidity. The diaphragm is not disproportionately affected by the dystrophic process compared with limb muscles.
Adult↗
Respiratory manifestations of rigid spine syndrome.
Thoracic abnormalities and respiratory muscle function were investigated in nine patients with rigid spine syndrome. A severe restrictive chest wall defect and limited mobility of the spine associated with clinically significant respiratory muscle weakness were present in all patients. Respiratory muscle strength and endurance were less than 60% of control values. Slight to moderate scoliosis was present in five patients and absent in four. Scoliosis appeared to have only a minor additional effect on respiratory muscle function. Six patients were emaciated, and one patient was underweight, but no relationship was seen between body mass index and respiratory muscle strength. Respiratory muscle function was more impaired in patients with hypoventilation than in normocapnic patients. Respiratory muscle involvement appears to be a significant feature of rigid spine syndrome, terminating in hypercapnic ventilatory failure in some patients.
Adolescent↗
The rigid spine syndrome: a vacuolar variant.
The rigid spine syndrome encompasses a number of disorders. We report 7 males and 2 females with this phenotype and a single, not previously reported, nosology. The salient muscle histological features were autophagic vacuoles, vacuoles containing capillaries, muscle spindle swelling, and type I fiber predominance. Disease onset was before age 6 years in all patients. Inheritance was probably autosomal recessive as siblings were affected in two families. Pulmonary function tests showed severely restricted ventilation, 3 patients required nocturnal ventilatory assistance, 2 patients had cor pulmonale, and mitral valve abnormalities were common. Serum CK levels were moderately elevated. EMG studies showed evidence of an active, chronic myopathy. The mean motor unit potential duration was statistically significantly shorter compared to controls in the triceps and anterior tibial muscles. Single fiber EMG "jitter" and evoked potential studies were normal.
Adolescent↗