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Biomedical subjects

D Jefferson

Publications and source records attributed to D Jefferson.

At least 19 recordsLinked to original sources

Patterns of mortality in patients with motor neurone disease.

OBJECTIVE: Motor neurone disease (MND) is a rapidly fatal condition with survival of less than 4 years. Patients can deteriorate quickly in the preterminal stages resulting in inappropriate resuscitation or admission to intensive care units (ICU) or accident and emergency (A & E). MATERIAL AND METHODS: We looked at patterns of mortality with emphasis on the place of death. A retrospective study was performed of all patients attending an MND clinic, who had died within a 10-year period. RESULTS: Of 179 patients (63 female), 81 patients (45%) died at home, in a hospice or in a nursing home. Sixty-five patients (36%) died in hospital (11 in ICU or A & E). Nine of the latter were previously known to have MND and six admissions were probably avoidable. Most ward patients died of respiratory causes and were treated conservatively. CONCLUSION: The proportion of patients dying in A & E or ICU was small but could have been reduced further. A number of those who died on the wards could probably have been managed conservatively at home. Older patients and those with bulbar disease had a poorer prognosis.

Adult↗

Relationship between supramaximal flow during cough and mortality in motor neurone disease.

The main function of cough is clearance of intrathoracic airways. A normal cough is characterized by a transient increase in expiratory flow above the maximal flow-volume loop envelope, known as cough "spikes". They may be absent in patients with motor neurone disease. The relationship between cough pattern, pulmonary function and survival was studied. Fifty-three patients were recruited (25 bulbar). Vital capacity, maximal inspiratory and expiratory mouth pressures and cough flow/volume curves were performed on all patients, and the presence or absence of spikes were recorded. The primary endpoints were mortality or initiation of ventilatory support over a period of 18 months. Thirty-five patients died over the 18-month period of the study (including the six who were started on noninvasive ventilation). Twelve of the 24 patients with spikes died compared to 23 out of 29 patients without spikes (p<0.05). Patients without spikes were more likely to be bulbar on clinical grounds (p<0.0001) and had poorer lung function. The results showed an association between the absence of cough spikes and increased mortality. However the main determinants of survival in motor neurone disease are age, vital capacity and inspiratory mouth pressure, and it remains to be shown whether regular monitoring of cough conveys any additional advantage.

Adult↗

Sniff nasal inspiratory pressure as a marker of respiratory function in motor neuron disease.

Vital capacity (VC) and maximum mouth pressures are often used to monitor respiratory function in motor neuron disease (MND), but require the use of a mouthpiece. Sniff nasal inspiratory pressure (SNIP) is a simple and reliable means of measuring inspiratory muscle strength; it does not involve the use of a mouthpiece and might therefore be better than VC or mouth pressures for assessing patients with bulbar disease. SNIP, maximum inspiratory (MIP) and expiratory mouth pressure (MEP), VC and arterial carbon dioxide tension (Pa,CO2) were measured in 59 consecutive patients attending a specialist MND clinic. Thirty-one had bulbar involvement on clinical grounds. Both SNIP and VC were inversely related to Pa,CO2 in nonbulbar patients only. Neither MIP nor MEP were related to Pa,CO2. The 10 patients with an elevated Pa,CO2 (>6 kPa) had significantly lower SNIP and VC than normocapnic patients. Sniff nasal inspiratory pressure can be used to monitor respiratory function in motor neuron disease. It is quick and easy for patients to perform, but otherwise appears to offer little advantage over vital capacity measurement. Patients with bulbar disease are often poor at performing sniff nasal inspiratory pressure manoeuvres, possibly because of upper airway collapse or inability to close the mouth completely during the manoeuvre.

Female↗

Medial epicondylectomy or ulnar-nerve transposition for ulnar neuropathy at the elbow?

We carried out a prospective randomised study comparing medial epicondylectomy with anterior transposition for the treatment of ulnar neuropathy at the elbow. The mean follow-up period was 4.5 years and we assessed the patients neurologically and orthopaedically. Neither procedure appeared to have a significant effect on elbow function. Our study showed better results after medial epicondylectomy; in particular patient satisfaction was higher than after ulnar nerve transposition. There were no significant differences in motor power or nerve-conduction rates and sensory fibres appeared to be more vulnerable to devascularisation.

Adult↗

Migraine angiitis precipitated by sex headache and leading to watershed infarction.

Vasospasm is a rare cause of cerebrovascular disease except following subarachnoid haemorrhage. We describe a woman who developed an explosive-type sex headache, followed by a series of severe migrainous headaches associated with fully reversible segmental cerebral arterial narrowing and dilatation, resulting in widespread infarction in cerebral arterial border zones. This led to transient loss of consciousness and multiple focal cortical deficits including blindness. She had a past history of migraine and a family history of both migraine and sex headaches. Similar cases have been reported in the literature under a variety of rubrics. We suggest that this newly recognized clinico-radiological syndrome is a migraine variant.

Adult↗

Idiopathic common peroneal nerve palsy--a review of thirteen cases.

Thirteen cases of idiopathic common peroneal nerve palsy were reviewed and the clinical presentation and course evaluated. Incomplete nerve palsy of unknown aetiology has a good prognosis and all the patients in this group made a full recovery without surgical intervention. Those presenting with a clinically and electrophysiologically complete lesion made no recovery, despite surgical intervention in two cases. It is suggested that these lesions can safely be treated conservatively as long as there is no identifiable cause.

Action Potentials↗

Inclusion bodies in motor cortex and brainstem of patients with motor neurone disease are detected by immunocytochemical localisation of ubiquitin.

Histological sections of cerebral motor cortex, brainstem, and spinal cord from 10 cases of clinically diagnosed motor neurone disease (MND) and 10 control cases were examined by conventional histology and immunocytochemical methods to localise ubiquitin. Intracytoplasmic inclusion bodies were identified in motor neurones of hypoglossal nuclei and appeared specific for MND. Similar inclusions were found in both large pyramidal cells and small neurones in the motor cortex, and were restricted to 4 cases having the amyotrophic lateral sclerosis form of MND with severe degeneration of corticospinal tracts. As reported in earlier studies, cellular inclusion bodies were identified in motor neurones of spinal cord from cases of MND but not in control material. Ubiquitin inclusions in motor neurones appear to be markers for the degenerative process causing neuronal loss in MND and there appears to be a close association between the anatomical location of inclusions and clinical manifestations of disease.

Aged↗

Ubiquitin gene expression in brain and spinal cord in motor neurone disease.

A restriction fragment of the coding region of a human ubiquitin gene has been used in Northern analyses of RNA prepared from human motor cortex and anterior horn region of cervical spinal cord. The analyses show that there is a substantial increase (approximately two-fold) in the expression of a polyubiquitin gene in motor cortex and spinal cord from patients with motor neurone disease compared to these tissues from control cases. Polyubiquitin gene expression in other organisms is associated with physical or chemical cell stresses. The data indicate that the primary stresses which result in the generation of ubiquitinated filamentous inclusion bodies in neurones in motor neurone disease also result in increased transcription of a gene coding for a polyprotein of ubiquitin.

Adult↗

A filamentous inclusion body within anterior horn neurones in motor neurone disease defined by immunocytochemical localisation of ubiquitin.

Using an immunocytochemical method to localise antibodies to ubiquitin, filamentous inclusion bodies were seen in spinal anterior horn neurones in cases of motor neurone disease (MND) but not in any control cases. These inclusion bodies appeared to be closely associated with classical Bunina bodies and immuno-electron microscopy suggested that they were based on arrays of straight 10-15 nm filaments together with some granular material. These observations link the protein ubiquitin with a chronic neurodegenerative disease and extend previous observations of a close association between filamentous inclusion bodies and ubiquitin. Ubiquitin-filament inclusions should be regarded as a new hallmark in the histological diagnosis of MND.

Adult↗

The comparative effects of ICI 118551 and propranolol on essential tremor.

1. The effects of the selective beta 2-adrenoceptor antagonist ICI 118551 on essential tremor, heart rate and blood pressure were compared with those of propranolol. 2. ICI 118551 (150 mg daily for 7 days) and propranolol (120 mg daily for 7 days) were about equally effective in reducing essential tremor (by about 40%) and were more effective than placebo. 3. When compared with the effect of placebo, propranolol reduced blood pressure and exercise heart rate whereas ICI 118551 had no significant effect on blood pressure and produced a small but significant reduction in exercise-induced tachycardia. 4. ICI 118551 may be useful in the management of essential tremor while having fewer cardiovascular side-effects than non-selective beta-adrenoceptor antagonists.

Adrenergic beta-Antagonists↗