[Selective immunoadsorption in neurologic complications of systemic lupus erythematosus]. / Selektive Immunadsorption bei neurologischen Komplikationen eines systemischen Lupus erythematodes.

Vasculitis of the nervous system is a rare cause of multifocal neurologic symptoms and may involve both the central and peripheral nervous systems. Typical symptoms include headache, encephalopathy with cognitive impairment and psychotic symptoms, epileptic seizures, and peripheral neuropathies. Here we report the case of a 71-year-old female presenting with Raynaud's syndrome and paresthesia of the feet. Several weeks later she was admitted to our hospital with a status epilepticus and complex partial seizures. On admission she had mild aphasia, distal paresis of the arms without sensory deficits, and disorientation with hallucinations. Cerebral MRI revealed small, multifocal infarctions in several arterial territories. Multiple cerebral artery stenoses were detected by ultrasound. Examination of the CSF was unremarkable. Serologic tests for autoimmune disorders detected Ro antibodies compatible with systemic lupus erythematosus or Sjögren's syndrome. A sural nerve biopsy revealed ischemic axonal neuropathy. During administration of i.v. methylprednisolone, the symptom progression stopped but dosages could not be tapered due to severe CNS symptoms (mental decline, disorientation, aphasia, hallucinations). Slow but sustained clinical improvement was achieved by immunoadsorption over 3 weeks followed by a combined high-dose immunosuppressive treatment with cyclophosphamide and prednisolone that paralleled a reduction in anti-Ro titers and normalization of cerebral blood flow velocities as detected by repeated transcranial Doppler sonography. Systemic vasculitis may present with multiple neurologic and psychiatric symptoms due to involvement of the central and peripheral nervous systems. After excluding systemic infection, immunosuppressive therapy should be started early. In our case a combination of high-dose methylprednisolone, immunoadsorption with elimination of Ro antibodies, and cyclophosphamide led to the patient's recovery.

Outcome after decompressive craniectomy in patients with severe ischemic stroke.

Decompressive craniectomy after space occupying infarction of the middle cerebral artery (MCA) tends to decrease mortality and increase functional outcome. The aim of this retrospective study was to evaluate mortality rates and functional outcome in our centre and to identify predictors of prognosis. The charts of 30 consecutive patients (6 women, 24 men, mean age 59.3 +/- 11.0 years) who underwent craniectomy after space occupying MCA-infarction from 1996 to 2002 were analyzed. Functional outcome was assessed by semistructured telephone interview as Barthel-Index, modified Rankin scale and extended Barthel-Index. Five patients (mean age 67.2 +/- 6.1 years) died within 5.2 +/- 2.4 days (range 2-8 days) after the first symptoms due to herniation. Nine patients (mean age 63.1 +/- 7.1 years) died 141.0 +/- 92.5 days (range 40-343) after stroke onset due to internal complications. 16 patients survived (mean surviving time 2.1 +/- 1.5 years, mean age 54.1 +/- 11.4 years). Mortality was related to age and the number of risk factors/comorbidity, and functional outcome was dependent on the number of risk factors/comorbidity. Our small observational, retrospective study suggests that hemicraniectomy in patients with space occupying MCA-infarction decreases mortality rate and increases functional outcome. Further randomized trials may prove useful to better define the indications, timing and prognosis for this procedure.

Bedside assessment of cerebral perfusion reductions in patients with acute ischaemic stroke by near-infrared spectroscopy and indocyanine green.

OBJECTIVE: To detect perfusion reductions in patients with acute cerebral infarcts using near-infrared spectroscopy (NIRS) with indocyanine green (ICG) as tracer. METHODS: Kinetics of an intravenous bolus of ICG were monitored by NIRS in 13 patients with acute infarction in the territory of the middle cerebral artery (mean (SD) age, 62.2 (13.0) years) and 12 controls (64.2 (9.1) years) at 2.8 (2.8) days after onset. NIRS optodes were placed bitemporally, with an interoptode distance of 4-5 cm. Absolute concentration changes in ICG were calculated. The following were assessed: time to peak, maximum ICG concentration, time interval between 0% and 100% maximum ICG concentration (interval), rise time (time between 10% and 90% ICG maximum), slope (maximum Delta ICG/interval), and blood flow index (BFI = maximum Delta ICG/rise time) of each hemisphere. Intraindividual differences were calculated between the two hemispheres. RESULTS: Patients with ischaemic stroke had increased time to peak (p<0.01), interval (p<0.01), and rise time (p<0.01), while maximum ICG concentration (p<0.03), slope (p<0.01), and BFI (p<0.01) were diminished at the site of infarction compared with the unaffected hemisphere. In stroke patients, intraindividual differences in time to peak (p<0.001), interval (p<0.001), rise time (p = 0.001), maximum ICG concentration (p<0.02), slope (p<0.001), and BFI (p<0.001) were greater than in the controls, with excellent sensitivity and specificity for Delta time to peak (100% and 100%, respectively) and Delta time interval (100% and 91.7%). CONCLUSIONS: Measurement of interhemispheric differences in ICG kinetics by NIRS detects perfusion reductions in patients with acute middle cerebral artery infarction. This non-invasive bedside test is rapid, repeatable, without major side effects, and avoids transportation of critically ill patients.

[Cerebral vasospasms with hemodynamic infarctions as a complication of HELLP syndrome]. / Zerebrale Vasospasmen mit hämodynamischen Infarkten als Komplikation eines HELLP-Syndroms.

Sinal thrombosis, intracerebral bleeding, cerebral edema, and cerebral vasospasm are typical neurological complications in pre- and eclampsia. Hemolysis, elevated liver enzyme, and low platelet (HELLP) syndrome is a rare, severe complication of pre-eclampsia. We present the case of a 33-year-old woman with HELLP syndrome. After a cesarean section, generalized epileptic seizure occurred and intubation was necessary due to sustained unconsciousness. Despite magnesium therapy, bilateral hemodynamic ischemic infarctions and narrowing of the circle of Willis were visible on MRI, and vasospasm of all large basal cerebral arteries was confirmed by cerebral angiography and transcranial Doppler sonography. During the following 2 weeks, the cerebral vasospasm resolved with application of high-dose methyl prednisolone and nimodipine. After extubation, the patient initially suffered from mild psychosyndrome and ataxia but recovered completely after 3 months. The HELLP syndrome may be complicated by cerebral vasospasm with subsequent hemodynamic strokes. Combined treatment with magnesium, corticosteroids, and nimodipine can be recommended. In our case, this regimen led to resolution of the cerebral vasospasm and complete clinical recovery.

Spontaneous oscillations in cerebral blood flow velocity give evidence of different autonomic dysfunctions in various types of headache.

OBJECTIVES: Our objectives were to determine if: (1) patients with migraine have B wave abnormalities in comparison to normal controls and patients with chronic tension headache and (2) patients with chronic tension headache have an imbalance in autonomic activity that is reflected in differences in Mayer wave activity in comparison to normal controls. BACKGROUND: B waves and Mayer waves are spontaneous oscillations in cerebral blood flow velocity with a frequency of 0.5 to 3 or 4 to 7 cycles per minute, respectively, and can be measured by transcranial Doppler sonography. There is experimental evidence that B waves are generated by certain brain stem nuclei which modulate the lumen of the small intracerebral vessels via monoaminergic nerve endings. In contrast, Mayer waves in cerebral blood flow velocity have no central generator but mirror the Mayer waves in arterial blood pressure which represent peripheral autonomic activity. Migraine may be attributed to a neurotransmitter imbalance in brain stem nuclei. Dysfunctions of the peripheral autonomic nervous system are known in patients with chronic tension headache. METHODS: Using bilateral transcranial Doppler monitoring of the middle cerebral artery B waves and Mayer waves were studied in 30 patients with migraine without aura, 28 subjects with tension-type headache, and 30 normal controls. Coefficient of variation as a quantitative parameter for amplitude of waves and the mean frequency were calculated from the envelope curves of the Doppler spectra. RESULTS: The coefficient of variation of B waves was higher in migrainous patients compared with patients with tension-type headache and normal controls (P<.05), indicating an increase in activity of brain stem nuclei in migraine only. Patients with chronic tension headaches had lower values for Mayer wave activity in comparison with normal controls (P<.05), a sign of an impairment of sympathetic activity. CONCLUSIONS: Our data support the dysfunction of the brain stem monoaminergic/serotonergic system in migraine. In contrast, patients with chronic tension headache have an autonomic dysfunction of peripheral origin presenting as a decrease of sympathetic activity.