Clinical management guidelines for subarachnoid haemorrhage. Diagnosis and treatment.

OBJECTIVE: To update the Spanish Society of Neurology's guidelines for subarachnoid haemorrhage diagnosis and treatment. MATERIAL AND METHODS: A review and analysis of the existing literature. Recommendations are given based on the level of evidence for each study reviewed. RESULTS: The most common cause of spontaneous subarachnoid haemorrhage (SAH) is cerebral aneurysm rupture. Its estimated incidence in Spain is 9/100 000 inhabitants/year with a relative frequency of approximately 5% of all strokes. Hypertension and smoking are the main risk factors. Stroke patients require treatment in a specialised centre. Admission to a stroke unit should be considered for SAH patients whose initial clinical condition is good (Grades I or II on the Hunt and Hess scale). We recommend early exclusion of aneurysms from the circulation. The diagnostic study of choice for SAH is brain CT (computed tomography) without contrast. If the test is negative and SAH is still suspected, a lumbar puncture should then be performed. The diagnostic tests recommended in order to determine the source of the haemorrhage are MRI (magnetic resonance imaging) and angiography. Doppler ultrasonography studies are very useful for diagnosing and monitoring vasospasm. Nimodipine is recommended for preventing delayed cerebral ischaemia. Blood pressure treatment and neurovascular intervention may be considered in treating refractory vasospasm. CONCLUSIONS: SAH is a severe and complex disease which must be managed in specialised centres by professionals with ample experience in relevant diagnostic and therapeutic processes.

Manometric correlations of anorectal dysfunction and biofeedback outcome in patients with multiple sclerosis.

OBJECTIVE: To evaluate clinical and manometric characteristics of multiple sclerosis (MS) patients with anorectal dysfunction (ARD) and their influence on biofeedback outcome. PATIENTS AND METHODS: Patients were clinically and manometrically studied and compared with controls. Patients were subsequently offered to initiate biofeedback manoeuvres to improve ARD. RESULTS: Fifty-two patients with ARD, 39 women, mean age 44.96 +/- 9.26 years, mean Expanded Disability Status Scale 4.13 +/- 1.72, were evaluated. Thirty-one patients had relapsing-remitting (RR), 16 secondary progressive and five primary progressive MS. ARD complaints were constipation (67.3%), double ARD (23.1%) and isolated incontinence (9.6%). The manometric study showed significant differences in patients compared with controls in maximal contraction pressures (98.1 +/- 44.2 mm Hg versus 152.05 +/- 66.9 mm Hg, P < 0.001) and anal inhibitory reflex threshold (92.9 +/- 63.4 mL versus 40.45 +/- 11.3 mL, P < 0.001). Maximal pressure was lower in progressive forms compared with RR forms (83.1 +/- 36.2 mm Hg versus 108.2 +/- 46.7 mm Hg, P < 0.05) in relation to higher disability. Patients with paradoxical contraction (PC) (35 patients, 67.3%) showed more manometric disturbances. From a total of 18 patients performing biofeedback, those reporting some improvement (six complete, two partial) had milder manometric abnormalities. CONCLUSIONS: The most frequent manometric abnormalities in our MS patients with ARD were alterations of maximal pressures, anal inhibitory reflex and PC. Biofeedback could be more useful in patients with lower disability and manometric alterations.

Periodic hyperthermia and abnormal circadian temperature rhythm in a patient with multiple sclerosis.

Disturbances of the central thermoregulation in patients with multiple sclerosis (MS) are not often reported. We describe a 45-year old patient with a 13-year history of MS, who developed a clinical picture of recurrent hyperthermia. MRI showed a bilateral involvement of the hypothalamus in the setting of diffuse white matter disease. Serial body temperature measurement for five consecutive months disclosed an inversion of the circadian temperature rhythm. The clinical presentation and MRI findings suggested abnormalities of the central thermal control in relation to MS widespread involvement of the central nervous system (CNS).

Valproate-induced hyperammonemic encephalopathy.

Valproate-induced hyperammonemic encephalopathy (VHE) is an unusual complication characterized by a decreasing level of consciousness, focal neurological deficits, cognitive slowing, vomiting, drowsiness, and lethargy. We have thoroughly reviewed the predisposing factors and their screening, the biochemical and physiopathological mechanisms involved, the different treatments described, and those that are being investigated. Etiopathogenesis is not completely understood, although hyperammonemia has been postulated as the main cause of the clinical syndrome. The increase in serum ammonium level is due to several mechanisms, the most important one appearing to be the inhibition of carbamoylphosphate synthetase-I, the enzyme that begins the urea cycle. Polytherapy with several drugs, such as phenobarbital and topiramate, seems to contribute to hyperammonemia. Hyperammonemia leads to an increase in the glutamine level in the brain, which produces astrocyte swelling and cerebral edema. There are several studies that suggest that treatment with supplements of carnitine can lead to an early favorable clinical response due to the probable carnitine deficiency induced by a valproate (VPA) treatment. Development of the progressive confusional syndrome, associated with an increase in seizure frequency after VPA treatment onset, obliges us to rule out VHE by screening for blood ammonium levels and the existence of urea cycle enzyme deficiency, such as ornithine carbamoyltransferase deficiency. Electroencephalography (EEG) is characterized by signs of severe encephalopathy with continuous generalized slowing, a predominance of theta and delta activity, occasional bursts of frontal intermittent rhythmic delta activity, and triphasic waves. These EEG findings, as well as clinical manifestations and hyperammonemia, tend to normalize after VPA withdrawal.