Autonomic dysre¯exia (AD) is an acute syndrome characterised by inappropriate and massive autonomic response that occurs in patients with spinal cord injury above the T6 level. Aims: to evaluate the incidence of AD during cystometry and the relationships with clinical and urodynamic features. Patients and methods: Forty-eight spinal cord injury patients were studied by neurological and urological examination and urodynamic evaluation with concurrent recording of blood pressure, heart rate and symptoms and signs of AD. Patients were considered to have AD if blood pressure reached values higher than 150/100 mmHg. Results: All the patients showed a signi®cant increase of both systolic and diastolic blood pressure, although only 20 showed pressure values higher than 150/100 mmHg (in seven of them without AD symptoms). AD was more frequent in cervical patients (P=0.034), but did not correlate with any other clinical features: sex ratio, age, disease duration, completeness of lesion, incidence of detrusor hyperre¯exia/are¯exia and detrusor-sphincter dyssynergia, voiding modalities, usage of anticholinergic drugs. In three patients blood pressure increase began when uninhibited contraction started, in 11 it was coincident with unhibited contraction peak and in the other six it appeared at maximum bladder capacity. Conclusions: (1) during urodynamic evaluation all the patients with lesion level above T6 showed signs of sympathetic stimulation, although only some showed dangerous blood pressure values; (2) the relationship between urodynamic data and dysre¯exia crisis shows that both the presence of detrusor uninhibited contractions and bladder distension are able to stimulate the crisis; (3) treatment with anticholinergic drugs is not sucient to prevent autonomic dysre¯exia starting from the bladder, unless it induces detrusor are¯exia. These patients are at risk of developing autonomic dysre¯exia following bladder distension.
BackgroundGoltz syndrome is a rare, genetic disorder mainly occurring in female patients.Case presentationThe case presented here is, to the best of our knowledge, the first description of the occurrence of lung parenchymal alterations in a young female patient affected by Goltz syndrome. Although pulmonary involvement is not known in patients affected by X-linked Goltz syndrome, the case here described is related to the even rarer autosomal form of the disease, as in this case. It is thus conceivable that in such different genetic setting the involvement of lung parenchyma may be unveiled through atypical emphysematous lesions.ConclusionThis report suggested - for the first time time - a rationale for a lung function and imaging screening in patients affected by Goltz syndrome at least in its autosomal form.
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