Mollaret's meningitis. A variant of recurrent hereditary polyserositis, both provoked by metaraminol.

Mollaret's meningitis is a rare condition with a characteristic clinical and cerebrospinal fluid picture. In many ways it resembles recurrent hereditary polyserositis (familial Mediterranean fever) in its natural history, pattern of attacks, and response to colchicine. Association of the two conditions has been reported, so far, in two patients only. In our patient the symptoms of both conditions were induced by a metaraminol provocative infusion. We have previously introduced this as a specific diagnostic and confirmatory test for recurrent hereditary polyserositis. The possibility that the two conditions represent different manifestations of a single disease is therefore strengthened.

Comparative trial of valproate sodium and clonazepam in chronic epilepsy.

A crossover comparative study of valproate sodium and clonazepam in the treatment of 32 adult epileptic patients receiving multiple drug therapy is described. Serum concentrations of other anticonvulsant drugs were unchanged by the addition of clonazepam. However, patients receiving high doses of other anticonvulsant drugs had lower serum concentrations of clonazepam (p less than .01). With valproate sodium, phenobarbital concentrations increased (P less than .05) in patients receiving phenobarbital but not significantly in patients receiving primidone. Phenytoin concentrations were reduced (P less than .05) during treatment with valproate sodium. Both drugs significantly reduced the frequency of minor seizures, with valproate sodium having the greater effect. However, it is important to monitor serum concentrations of other anticonvulsant drugs during treatment with valproate sodium since changes in these may influence seizure control or cause side effects.

Rapid diagnosis of central nervous system brucellosis by ELISA.

The diagnosis of central nervous system (CNS) brucellosis was made by determining Brucella-specific IgG, IgM and IgA in cerebrospinal fluid (CSF) specimens, using an enzyme-linked immunosorbent assay (ELISA) method. A total of 68 CSF specimens including 10 from patients with brucellosis of the nervous system, 4 with Brucella infection but without CNS involvement, 38 with meningitis other than due to Brucella and 16 with no meningitis were studied. Of the 10 CSF specimens from patients with CNS brucellosis, Brucella-specific IgG was detected in all 10, IgM in 7 and IgA in 8. The 10 patients were also seropositive for anti-Brucella IgG and IgM, and 9 were seropositive for IgA. No Brucella-specific IgG, IgM or IgA was detected in the CSF of 4 patients with brucellosis but without CNS involvement, even though the blood of these patients had high titres of anti-Brucella IgG, IgM and IgA. In addition, none of the CSF specimens from the 38 patients with meningitis other than due to Brucella, or from the 16 patients without meningitis, had detectable anti-Brucella antibodies. This study indicates that the Brucella ELISA on CSF is a reliable, rapid, sensitive and specific assay in the diagnosis of CNS brucellosis.

ELISA versus routine tests in the diagnosis of patients with systemic and neurobrucellosis.

Sera from patients in different stages of brucellosis as well as sera and cerebrospinal fluid (CSF) from patients with central nervous system (CNS) brucellosis and controls, were tested by ELISA for Brucella-specific IgG, IgM and IgA. The results were compared with culture findings, micro-agglutination (MA), slide agglutination with Rose Bengal (RB), and Brucella melitensis stained antigens (SA). In sera of patients with acute brucellosis (296), ELISA was positive for IgM (100%), IgG (97%) and IgA (98%), and comparable results were found in sera of patients with subacute brucellosis (44): IgG (100%), IgM (86%) and IgA (100%). However, in patients with chronic brucellosis (40), IgG and IgA were consistently positive (100%) while IgM was only positive in 33% of their sera. The MA and RB showed similar results, being more positive in patients with acute (98%) and subacute (84%) than in chronic (61%) brucellosis. The SA and culture showed significantly lower positive results. In the CSF of patients with CNS brucellosis (45), ELISA was positive in 100%, 20% and 85% for IgG, IgM and IgA, respectively, compared to 13% positive by culture, 25% by MA and 22% by RB. ELISA was negative in the CSF specimens from patients with brucellosis without CNS involvement (66), or meningitis other than Brucella (62), and no meningitis (144). Thus, ELISA with its IgG, IgM and IgA profiles is the test of choice in the diagnosis of patients with brucellosis, especially those with chronic or CNS infection.

New variant Creutzfeldt-Jakob disease.

Since the report of new variant Creutzfeldt-Jakob Disease (nvCJD) in humans last year, the search was on for direct evidence to link the condition to Bovine Spongiform Encephalopathy (BSE). The first case nvCJD was noted 10 years after the recognition of BSE in UK cattle. A direct link is now established. There are, however, some 'protective' mechanisms, the most important of which are the inefficiency of the gastric route of introducing the infected material, the species barrier and genetic 'predisposition'. None of these is full proof and time will tell whether feeding ruminants with their own species will turn out to be a major public health disaster?

Brainstem auditory evoked potential (BAEP) abnormalities in brucellosis.

Twelve patients with neurobrucellosis and 17 patients with systemic brucellosis without neurological involvement underwent a brainstem auditory evoked potentials (BAEP) study. All neurobrucellosis patients (100%) showed abnormalities in their BAEP recordings, suggestive of brainstem lesions at various levels. On the other hand, only 5 (29%) of the 17 patients with systemic brucellosis had mild unilateral BAEP abnormalities, while the remaining 12 had normal responses. Comparison of pooled data between the systemic brucellosis and neurobrucellosis groups showed highly significant differences in all BAEP parameters. The recording of BAEP is thus considered a sensitive supplementary method to reveal CNS lesions in patients with neurobrucellosis.

The role of folate deficiency in the development of peripheral neuropathy caused by anticonvulsants.

To investigate the role of folate deficiency in neuropathy caused by anticonvulsants, electrophysiological studies of peripheral nerve function were carried out on 29 epileptic patients on long-term anticonvulsant therapy. All but three patients showed abnormalities in one or more electrophysiological measurements, the main abnormality being in amplitude of sensory nerve action potential--this was reduced or absent in 76% of patients. All patients had low concentrations of folate in serum and CSF, these being below the normal ranges in 19 patients. These 19 patients were treated with folate, either 5-formyltetrahydrofolate (10 patients) or folic acid (9 patients), over a period of one month. After therapy all patients had normal levels of folate in serum and CSF, slightly higher levels in CSF being obtained in those receiving 5-formyltetrahydrofolate. Folate therapy significantly reversed abnormalities in motor and sensory nerve distal latencies; the effect was greater with 5-formyltetrahydrofolate, apparently because this produced higher CSF folate concentrations than folic acid. We conclude that folate deficiency may be involved in the development of peripheral neuropathy due to anticonvulsants.

Brainstem auditory evoked potentials in Wilson's disease.

Twelve patients with Wilson's disease, aged 11-25 years, underwent brainstem auditory evoked potential (BAEP) study. The results were correlated to clinical, neuroradiological and laboratory data. Ten had prominent to severe neurological manifestations, suggestive of involvement of one or several CNS structures, and 2 were neurologically free. All had evidence of abnormal copper metabolism, and 8 had CT scan evidence of brain atrophy, or hypodense areas in basal ganglia, or both. The 2 patients without neurological manifestations as well as one with neurological signs had normal BAEP. One patient with neurological signs had increased N1 latency due to cochlear hearing loss, but normal interpeak intervals, while 8 of 10 patients with prominent neurological symptoms and signs had abnormal BAEPs (prolongation of NIII-NV interpeak interval). The value of NIII-NV interpeak interval correlated with the number of different neurological signs (neurological score) attributable to involvement of different CNS structures (r = 0.64 at P = 0.001). Abnormal BAEPs do not seem to be an early finding in Wilson's disease.

Surgery in combined pulmonary and neural cryptococcosis.

A 39-year-old previously healthy patient with combined pulmonary and neural cryptococcosis was successfully managed by pulmonary resection and antifungal chemotherapy. The pulmonary mass was removed under cover of miconazole and 5-fluorocytosine even though the patient had concomitant active meningitis. He recovered dramatically after the surgical procedure and remained well 2 years later. We suggest that, as in our patient, early removal of a pulmonary cryptococcal mass under antifungal cover may in other similar patients obviate the need for protracted antifungal therapy and lead to an excellent response of the neural cryptococcosis.

Spontaneous intracranial hypotension: clinical and magnetic resonance imaging characteristics.

The syndrome of spontaneous intracranial hypotension (SIH) is an uncommon cause of postural headache. We describe three patients with classical low pressure headache associated with low CSF pressure, one of whom presented with sudden deafness and another with a unilateral VIth nerve palsy. Initial magnetic resonance imaging (MRI) scans revealed bilateral diffuse subdural fluid collections in all three cases. Follow up MRI scans performed on two patients at 6 months demonstrated partial resolution of the subdural collections but persistent striking meningeal enhancement despite clinical recovery. These findings differ from previous reported cases wherein clinical resolution of postural symptoms was preceded or closely followed by resolution of the MRI changes.

Congenital malformations before and after the onset of maternal epilepsy.

A total of 222 pregnancies in 56 mothers were studied, 48 pregnancies occurred in 17 mothers before the onset of epilepsy (Group 1). The same group went on to develop epilepsy, receive anticonvulsants and further conceive 43 pregnancies (Group 1a). In the other 39 mothers 131 pregnancies occurred after the onset of epilepsy and while receiving anticonvulsants (Group 2). The incidence of major congenital malformations among live births was significantly higher in Group 2 (14.4%) when compared with all pregnancies in Groups 1 and 1a (1.2%), P less than 0.025 and in Group 1 alone (2.2%) P less than 0.05. There was no significant difference between Groups 1 and Group 1a. Age at first pregnancy in Group 1 was 18.9 years +/- 3.7 SD, which was significantly younger than Group 2, 21.7 years +/- 5.1 SD, p less than 0.05. The age of onset of epilepsy was significantly younger in Group 2 than in Group 1, 15.1 years +/- 6 SD vs 23.3 years +/- 4.6 SD, p less than 0.001. The duration of epilepsy was significantly longer in Group 2 compared with Group 1, 13.8 years +/- 8.7 SD vs 6.1 years +/- 2.6 SD, p less than 0.001. Patients on polytherapy had a significantly higher risk of major malformations compared to those on monotherapy, 20.2% vs 1.3%, p less than 0.001. Our data suggest that epileptic mothers with the highest risk of having offspring with major malformations were those with a long duration of epilepsy combined with exposure to multiple anticonvulsant drugs.

Giant cell arteritis presenting as oculomotor nerve palsy with pupillary dilatation.

Acute complete oculomotor palsy with headache is a classical presentation of an extrinsic compression most commonly due to a posterior communicating artery aneurysm. We present a patient with such a presentation but with histologically proven giant cell arteritis. This possibility should be considered especially in the elderly to avoid complications and the need for angiography.

Training of Kuwaiti medical doctors and their specialty choices: sociocultural impact.

Kuwait has a comprehensive and well-structured health care system. However, to deliver such care the country relies on expatriate medical doctors. This paper attempts to discuss the sociocultural influences on the training of Kuwaiti medical doctors and their specialty choices in order to gauge future requirements of the Kuwaiti Health Services.

Progressive myoclonic ataxia without ragged red fibres: Unverricht-Lundborg disease vs Ramsay Hunt syndrome.

We describe eight patients from three families presenting with myoclonus, ataxia, infrequent seizures and minimal intellectual impairment. All were Arabs from different parts of the Arabian peninsula. The new consensus on terminology, genetic and clinical definition of Baltic myoclonus, Ramsay Hunt syndrome and Unverricht-Lundborg disease suggests that our group are best categorised under the term of progressive myoclonic ataxia of the Unverricht-Lundborg type. Moreover, this report reinforces the existence of this syndrome outside Scandinavia.

Experience with neurocysticercosis in the UK: correct diagnosis and neurosurgical management of the small enhancing brain lesion.

Neurocysticercosis is a major cause of epilepsy and other neurological morbidity in endemic areas of the world but is exceptionally rare in the West. We have recently had experience of eight patients with this condition, seven presenting with epilepsy and single or multiple small, enhancing parenchymal lesions and one with hydrocephalus caused by a midbrain lesion. One lesion was stereotactically excised after it persisted, but in five other cases spontaneous cyst resolution was observed during expectant management with anticonvulsants. Two patients with multiple lesions were referred to us for further management but were free of active infection. Recent studies show that neurocysticercosis may often be diagnosed based upon the clinical, epidemiological and radiological features. Spontaneous cyst resolution is to be expected in this condition and suspected patients should be carefully observed and surgery avoided. We believe that this disease presents more commonly than has been appreciated in the UK and propose a protocol for management.

Neurological presentation of neuro-Behçet's syndrome: clinical categories.

In an attempt to categorize the diverse neurological manifestations of Behçet's syndrome, a group of 10 patients was prospectively studied; the diagnosis of Behçet's syndrome was not known in any of them prior to their neurological presentation. A reasonably clear distinction can be made to divide the manifestations into three general categories with some overlap. Category I includes patients with increased intracranial pressure with or without cerebral venous sinus thrombosis. Category II are those presenting with a stroke. Category III are those patients who present with spinal cord involvement and CSF pleocytosis. Brainstem involvement was noted independently of the group. In general, patients with Neuro-Behçet's syndrome showed little ocular involvement. The course of the illness both in morbidity and mortality tended to be better in category I, and mortality was only seen in category II patients. In populations susceptible to the disease, the clinical categorisation proposed may alert the clinician to the possibility of neuro-Behçet's syndrome whenever patients with pseudotumour cerebri, dural sinus thrombosis, stroke in the young, unexplained myelitis or possible multiple sclerosis are encountered.

Metabolism of immunoglobulin A, lymphocyte function, and histocompatibility antigens in patients on anticonvulsants.

Low serum IgA levels were found in patients taking phenytoin, together with evidence of depressed T cell function. There was no correlation between the dose or the serum level. A correlation was found, however, with HL-A status, patients with a low IgA showing increased frequency of HL-A2. It is suggested that epileptic patients with HL-A2 status are likely to develop IgA deficiency when given phenytoin.

Internal carotid artery occlusion in a young female with mitral valve prolapse.

A healthy 28 year old housewife presented with sudden right hemiplegia and aphasia. No predisposing factors could be ascertained apart from posterior cusp mitral value prolapse on two dimensional echocardiography. Extensive investigations confirmed the presence of cerebral infarction and persistent occlusion of the left internal carotid artery near its origin.