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Biomedical subjects

B D Youl

Publications and source records attributed to B D Youl.

31 records · Page 2Linked to original sources

Neurological deterioration in young adults with phenylketonuria.

7 patients with phenylketonuria who developed neurological disability in adolescence or early adult life are described. 4 had been diagnosed by routine neonatal screening and started a low phenylalanine diet in infancy. 3 were diagnosed in early childhood because of developmental delay, and then started dietary treatment. Dietary control deteriorated in later years and was withdrawn in mid to late childhood. The late neurological deterioration cannot be directly ascribed to poor compliance with or cessation of dietary treatment in this small, retrospective study--but other likely causes have been excluded and 2 patients showed a striking clinical improvement when a strict diet was resumed. Serial magnetic resonance images from one of these patients show abnormalities that appeared after cessation of dietary treatment and resolved after diet was resumed. If these findings are confirmed, strict dietary control into adult life would be indicated for at least some patients with phenylketonuria.

Adult↗

[Headaches after lumbar puncture and their treatment].

The principle mechanism of post lumbar puncture headache is the leakage of CSF from the dural puncture site. This leads to a state of low sub-arachnoid pressure, causing traction on pain-sensitive intracerebral structures. The operator can minimise the risk of headache by selecting a needle of small diameter and ensuring that the bevel is aligned parallel to the longitudinal fibers of the dura. Current evidence suggests that the patient may be allowed to mobilise immediately. Traditional therapies such as bed rest and rehydration are usually ineffective when headache is severe. If this is the case, and the diagnosis is certain, a blood patch procedure can be performed, and repeated if necessary.

Headache↗

Three cases of spontaneous extracranial vertebral artery dissection.

We describe three cases of extracranial vertebral artery dissection that are unusual in both their modes of presentation and their associations with other pathologic conditions. The first patient had Marfan's syndrome and migraine; his dissection was asymptomatic and was diagnosed by chance at the time of repeat angiography following a previous internal carotid artery dissection. The second patient had systemic lupus erythematosus and presented with a subarachnoid hemorrhage attributed to an intracranial vertebral artery dissection by the demonstration of an extracranial dissection. The third patient had a minor basilar artery stroke in which dissection had occurred beside a congenital hemivertebra deformity.

Adult↗

The occurrence of oligoclonal IgG in tears from patients with MS and systemic immune disorders.

Using isoelectric focusing (IEF) and immunoperoxidase staining of proteins transferred to nitrocellulose membranes, we have examined the IgG band pattern in tears and matched serum and CSF specimens of 28 patients with MS, 4 patients with optic neuritis (ON), 30 individuals with systemic, inflammatory, or other neurologic diseases, and 5 patients with tension headache. We found no evidence of positive oligoclonal IgG in tears in any MS or ON patients, while 10 out of 16 cases with systemic immune disorders or infections of the CNS had positive tear oligoclonal bands. We are thus not able to support the hypothesis that tears from MS patients reveal abnormalities in their humoral immune response.

Adult↗

Three cases of craniopharyngioma showing optic tract hypersignal on MRI.

Three patients presenting with visual failure had MRIs with hypersignal extending from the region of the optic chiasm along both optic tracts in 2 cases, and along 1 optic tract in the 3rd. In all patients intrinsic tumor of the chiasm was the most likely diagnosis based on MRI appearances, but all 3 had craniopharyngioma.

Adult↗

Migraine--a risk factor for dissection of cervical arteries.

A case control study technique was employed to test for an association between non-traumatic cervical artery dissection and several possible risk factors. A significant positive association was shown with migraine, independent of type and treatment regimen. No such relationship was found with smoking history, hypertension or past oral contraceptive use. There was, however a significant and independent association with current oral contraceptive use.

Adult↗

[Neurological manifestations of Behçet's disease].

Characterised classically by the association of buccal and genital ulceration and uveitis with hypopyon, Behçet's disease has many other manifestations, amongst which the neurological ones (often referred to as Neuro-Behçet) are important in view of their frequency and their gravity. Anatomically, it produces a subacute haemorrhagic and necrotising meningo-encephalitis, which most typically effects the hypothalamus and brainstem. Clinically, there is extreme polymorphism, central manifestations being the most frequent: seizures, organic brain syndromes, disorders of consciousness, aphasia, hemiplegia, cranial nerve palsies, pseudobulbar and extrapyramidal syndromes and meningism. The peripheral nerves and muscles are rarely affected. Alongside Neuro-Behçet per se, attention has recently been directed to the various cerebro-vascular manifestations, dominated by venous thrombosis. A review of the principal neurological manifestations is given, with comment on anatomico-pathological aspects, clinical presentation, investigational techniques, diagnostic difficulties, prognosis, and treatment.

Behcet Syndrome↗

Gowers' shorthand.

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England↗