Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Apr 2004
Reliability of self-reported diagnoses in patients with neurologically unexplained symptoms.
Patients with neurologically unexplained symptoms (NUS) often have a previous history of other medically unexplained symptoms. A past history of such symptoms can help make a positive diagnosis of a somatoform or affective disorder, and enable appropriate management strategies. However, information on past medical diagnoses is primarily obtained from patient interviews and may be inaccurate, particularly in patients with NUS. ⋯ Reported previous diagnoses should not be taken at face value when the current differential diagnosis includes a functional/somatoform neurological syndrome, particularly if the list of past medical diagnoses is long. Confirmation of previous diagnoses from alternative sources may contribute to a diagnosis of somatoform disorder, allowing appropriate management strategies for the current (and past) complaints to be initiated.
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J. Neurol. Neurosurg. Psychiatr. · Apr 2004
Case ReportsLate onset MLD with normal nerve conduction associated with two novel missense mutations in the ASA gene.
Metachromatic leukodystrophy (MLD) rarely has its clinical onset in young adults, with a combination of cognitive and behavioural symptoms and peripheral neuropathy. Here we present an exceptional case with very late onset at 42 years of age and no clinical or neurophysiological sign of peripheral neuropathy. Molecular analysis revealed compound heterozygosity for two novel missense mutations affecting conserved residues in the arylsulphatase A (ASA) sulphatase and carboxyterminal domains, resulting in an 89% loss of enzymatic activity. This case indicates that MLD needs to be considered in the differential diagnosis of very late onset white matter diseases, even if not accompanied by peripheral nerve involvement.
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J. Neurol. Neurosurg. Psychiatr. · Apr 2004
Percutaneous radiological gastrostomy: a safe and effective method of nutritional tube placement in advanced ALS.
Enteral nutrition may be required in amyotrophic lateral sclerosis (ALS), and is usually achieved by percutaneous endoscopic gastrostomy (PEG). As PEG is not indicated in patients with severe respiratory impairment, an alternative is percutaneous radiological gastrostomy (PRG), involving air insufflation into the stomach under fluoroscopic guidance for tube insertion. ⋯ PRG appears to be safer than PEG in ALS patients with moderate or severe respiratory impairment, and is followed by a longer survival.
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J. Neurol. Neurosurg. Psychiatr. · Apr 2004
Cognitive impairment and functional outcome after stroke associated with small vessel disease.
Although stroke associated with small vessel disease (SSVD) can induce both motor and cognitive impairment, the latter has received less attention. We aimed to evaluate the frequency of the varying severity levels of cognitive impairment, the determinants of severe cognitive impairment, and the association of cognitive impairment with functional outcome after SSVD. ⋯ Half of the patients with SSVD complained of varying severity of cognitive problems 3 months after stroke. Pre-stroke cognitive decline and previous stroke predict severe cognitive impairment post stroke. Stroke severity and executive dysfunction contribute most to a poor functional outcome.
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J. Neurol. Neurosurg. Psychiatr. · Apr 2004
Cognitive changes after epilepsy surgery in the posterior cortex.
The relationship between the posterior cortex and cognitive functions is still a relatively open field. There are no studies on populations in which functions of posterior structures were examined by a standardised neuropsychological examination before and after posterior resections. Changes in cognitive performance are regularly observed after epilepsy surgery in the temporal lobe. However, information about neuropsychological impairments after resections in the posterior cortex is poor, owing to the relatively low proportion of cortical resections in this area. ⋯ Functions of posterior areas could be described by standardised neuropsychological measures. Posterior regions contribute to explicit attentional and visuoconstructional abilities. Epilepsy surgery in the posterior cortex bears no risk for substantial decline in general cognition although some discrete impairment in performance intelligence may occur.