Papilloedema secondary to a spinal paraganglioma.

An asymptomatic 72-year-old man presented with bilateral papilloedema. Cranial CT imaging was normal, but lumbar puncture found an opening pressure of 320 mmH2O (120-250) with raised cerebrospinal fluid protein, increased red blood cells and xanthochromia. MR scan of spine showed a cauda equina tumour, histologically defined as a paraganglioma. The papilloedema resolved after surgery.

Parkinsonism associated with obstructive hydrocephalus due to idiopathic aqueductal stenosis.

Two cases of parkinsonism after recurrent obstructive hydrocephalus due to idiopathic aqueductal stenosis are reported. In both patients an extrapyramidal syndrome was noted in the absence of contemporaneous evidence of hydrocephalus or shunt failure. One of the patients underwent a shunt operation, but showed no clinical improvement. However, both patients improved after the administration of dopaminergic therapy. The seven previously reported cases of this syndrome were reviewed and it is concluded that the prognosis of the parkinsonism is good, usually with total, or near total, resolution. It is recommended that if a patient with idiopathic aqueduct stenosis develops hydrocephalus or evidence of shunt malfunction in association with acute parkinsonism their shunt should be replaced. If there is no evidence of hydrocephalus or shunt malfunction they should initially be treated with domaminergic medication.

New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests.

BACKGROUND: In April, 1996, ten cases of Creutzfeldt-Jakob disease (CJD) with an apparently new clinicopathological phenotype were published and it was suggested that these new variant cases (nvCJD) might be causally linked to bovine spongiform encephalopathy (BSE). There have now been 21 cases of nvCJD in the UK and one case in France. We report clinical features and diagnostic test results of the first 14 cases of nvCJD in the UK. METHODS: Case ascertainment of CJD was mainly by direct referral from neurologists and neuropathologists. Clinical and investigate details were obtained by interview with patients' relatives and by examination of case notes. Ten cases in this report were examined while alive. Prion protein (PrP) gene analysis was carried out with informed consent from the patient or from a relative. The diagnosis of nvCJD was established histologically. FINDINGS: Eight cases were women. Mean age at onset of symptoms was 29 (16-48) years and the median duration of illness was 14 (9-35) months. All patients had early psychiatric symptoms, most often depression, and 13 were seen by a psychiatrist early in the clinical course. Eight patients developed early sensory symptoms which were persistent and often painful. Neurological signs, including ataxia and involuntary movements, developed in all cases and towards the end of the illness, most had akinetic mutism. The electroencephalogram was abnormal in most patients but typical periodic complexes of CJD were not seen in any case. Cerebral imaging was usually normal or showed non-specific abnormalities; in two cases magnetic-resonance imaging scans showed high signal in the thalamus. INTERPRETATION: Clinical features in these cases are similar and relatively distinct from other forms of CJD, suggesting that this is a new clinical phenotype consistent with a single strain of infectious agent. There is, however, some overlap with atypical cases of sporadic CJD, and the diagnosis of nvCJD remains dependent on neuropathological confirmation.

Bilateral lambda light chain amyloidomas of the trigeminal ganglia, nerves and roots.

We describe a patient who developed chronic bilateral trigeminal neuropathy that was found at autopsy to be due to lambda light chain amyloidosis involving the trigeminal nerves, ganglia and roots bilaterally, as well as part of the intrapontine course of the trigeminal nerve fibres. No amyloid was found elsewhere in the nervous system or systemically. Review of previous reports indicates that the clinical features of trigeminal amyloidosis are quite stereotyped, with initial trigeminal neuralgia or dysaesthesiae, and subsequent development of facial anaesthesia and weakness of muscles of mastication. The disorder is usually unilateral but may rarely, as in the present case, occur bilaterally.

Computerised tomography scans of the head in a district general hospital.

We report the results of a retrospective study of all computerised tomography (CT) brain scans of adult patients carried out over a 10-week period, based on radiological and clinical records. The commonest scan results were normal (51%) and stroke (26%), with an overall diagnostic accuracy of 43/80 (54%). On the basis of a retrospective blind survey by a neurologist, 42% of scans were thought not to be indicated. The results indicate the need for: guidelines on the use of CT scanning in stroke. Emphasis of current guidelines on the use of CT scanning in head injury. Neurological assessment of some suspected diagnoses before scanning.

Facial palsy.

Facial palsy, particularly Bell's palsy, is a common neurological problem. This article attempts to provide a practical guide to diagnosis and treatment. The causes of facial palsy are discussed according to the site of the lesion and the associated signs. The prognosis and management of Bell's palsy is reviewed in detail.

Stiffman syndrome: a rare paraneoplastic disorder?

An unusual case of the stiffman syndrome, associated with an oat cell carcinoma of the bronchus, is reported. Pathological examination showed that it was due to an encephalomyelitis similar to that seen in paraneoplastic disorders. This suggests that atypical cases of the stiffman syndrome may occasionally be paraneoplastic.

The relationship between intracranial pressure and tympanic membrane displacement.

Transmission of intracranial pressure (ICP) to the perilymph of the cochlea may occur via the cochlear aqueduct and possibly other routes. Indirect measurement of perilymphatic pressure may be investigated by observing tympanic membrane (TM) displacement during stapedial reflex contraction. In a previous study we investigated the effects of changes in ICP on perilymphatic fluid pressure in three patients who underwent ventriculo/lumbar-peritoneal shunt operations. The TM displacement technique proved extremely sensitive and revealed marked changes in cochlear fluid pressure brought about by changes in ICP (Marchbanks et al., 1987). The study has been extended to 58 patients with hydrocephalus, intracranial tumours and other neurological conditions associated with abnormal ICP. Significant differences in the TM displacement were found between patients with raised and normal ICP. We have shown that changes in ICP can affect the hydrostatic pressure of the cochlea and influence the peripheral auditory system. The finding that ICP can be correlated with TM displacement strengthens the association between an abnormal TM displacement and abnormal cochlear hydrostatic status, irrespective of cochlear aqueduct patency. We suggest that the TM displacement technique provides a useful non-invasive method for the assessment of perilymphatic fluid pressure.

Mean intracranial pressure monitoring by a non-invasive audiological technique: a pilot study.

Intracranial pressure is normally transmitted to the perilymph of the cochlea via the cochlear aqueduct. The relationship between perilymphatic pressure, indirectly measured by tympanic membrane displacement, and mean intracranial pressure defined either clinically or by direct measurement has been examined in 58 patients (aged 5-77 years), with hydrocephalus, benign intracranial hypertension, intracranial tumours, subarachnoid haemorrhage and head injuries. The most consistent results were obtained in young patients with hydrocephalus and benign intracranial hypertension. However, the technique was not suitable when the stapedial reflex was absent as a result of middle ear/brainstem dysfunction and did not reflect intracranial pressure when the cochlear aqueduct was not patent. This pilot study suggests that the tympanic membrane displacement technique may provide a useful non-invasive method for serial monitoring of intracranial pressure in young patients with hydrocephalus or benign intracranial hypertension.

Lyme disease: prevalence and clinical importance of Borrelia burgdorferi specific IgG in forestry workers.

41 forestry workers, who had a high occupational risk of tick-bites, were screened for antibodies to Borrelia burgdorferi by ELISA and western blotting techniques, and questioned about possible symptoms of Lyme disease. Antibodies were detected in 10 of the 40 men who had been bitten by ticks. Definite symptoms of Lyme disease, in the form of erythema migrans, were reported by only 2 workers and none had a history of neurological illness.