Reversible cerebral vasoconstriction syndrome

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Classification according to ICD-10
I67.8 Other specified cerebrovascular diseases
ICD-10 online (WHO version 2019)

In reversible cerebral vasoconstriction syndrome (RCVS, also Call Fleming Syndrome ), constriction (contraction of the muscles) of cerebral vessels leads to severe headaches ( annihilating headaches ) with or without other neurological abnormalities, such as convulsions , strokes or subarachnoid hemorrhage . The symptoms pass within three months.

A number of names have already been used for the syndrome in English: Isolated benign cerebral vasculitis, acute benign cerebral angiopathy, reversible cerebral segmental vasoconstriction, call syndrome, call Fleming syndrome, CNS pseudovasculitis, benign angiopathy of the CNS, post - partum angiopathy, migraine angiitis, migrainous vasospasm, primary thunderclap headache, cerebral vasculopathy, vasospasm in fatal migrainous infarction.

Epidemiology

So far, RCVS has been reported in patients between 10 and 76 years of age, but it mostly occurs around the age of 40, especially in women. The exact frequency of the disease is unknown, but it does not appear to be a rare disease.

The syndrome occurs more frequently after childbirth , with eclampsia , after taking adrenergic or serotonergic medication and when using cocaine or amphetamines .

Symptoms

The main symptom of RCVS is headache . These usually begin as thunderclap headache ( thunderclap headache ). This occurs suddenly and leads to a very severe headache within a minute. These usually start at the back of the head and over time expand diffusely over the entire head. Common accompanying symptoms are agitation , confusion, a tendency to collapse , nausea , vomiting , photophobia and sensitivity to noise. The annihilation headache can either go away within minutes or after a few days. Typically, the headache with RCVS lasts a few hours, but there are usually multiple headache attacks within the first four weeks, with moderate headache between attacks.

Other possible symptoms are focal failures, usually for a few hours, and epileptic seizures , which usually only occur once.

The most important complications are ischemic or hemorrhagic strokes, which can lead to permanent damage.

The most important differential diagnoses are subarachnoid hemorrhage , tears in the walls of the neck vessels ( dissection ) and the very rare primary angiitis of the central nervous system .

Diagnosis

The laboratory tests (such as blood count , signs of inflammation , rheumatoid factor , antibodies , urine test) are normal in most cases, sometimes there may be slight deviations in the CSF test (increased leukocytes , erythrocytes , increased protein ). Also in the imaging of the brain there are no deviations from the norm apart from diffuse vasoconstriction in the cerebral angiogram . In computed tomography (CT) and magnetic resonance imaging (MRI) can only sometimes (subarachnoid or intracerebral hemorrhage, cerebral infarction, cerebral edema) infarcts due to the RCVS see.

When diagnosing it, it is most important to always think of RCVS in the case of annihilating headaches lasting several days, convex subarachnoid hemorrhages and strokes without a clear cause. Then a transcranial Doppler and indirect angiography (using CT or MRI) should be done to identify the bottlenecks caused by the vasoconstrictions . However, the picture can change significantly within a few days, especially in the first few weeks, often no deviations are visible. The catheter angiography , although sensitive, but leads in nine percent of cases transient ischemic attacks.

Diagnostic criteria for reversible cerebral vasoconstriction syndrome:

  • Acute and severe headache (often annihilating headache ) with or without focal deficits or seizures
  • further attacks without new symptoms within one month of the onset of symptoms
  • segmental vasoconstriction of cerebral arteries in the angiography visible
  • no evidence of aneurysmal subarachnoid hemorrhage
  • Little or no deviations in the CSF analysis (protein <100 mg / dL; <15 leukocytes / µl)
  • complete or as far as possible normalization of the angiography within twelve weeks of the onset of symptoms

therapy

Guidelines for the therapy of RCVS are still pending. However, early diagnosis plays an important role in proper treatment. Therefore, both patients with visible vasoconstrictions and patients with a suitable clinic, suitable imaging, lack of evidence of other causes and initially normal angiogram should be treated with symptomatic therapy.

This includes eliminating factors that could worsen vasoconstriction:

  • Stress , sexual activity, exercise, and other physical work, and other triggers for headaches
  • Vasoactive drugs

Further therapy consists in the administration of analgesics , benzodiazepines and, in the case of seizures, anti- epileptic drugs . The patient's blood pressure must be monitored; in severe cases, monitoring must be carried out in an intensive care unit. A hypertension should hypertension after a treatment according to the guidelines of the stroke carried out taking care to keep in mind that a hypotension at RCVS is worse than the hypertension.

The vasoconstriction can be resolved , if at all, with nimodipine , verapamil, or magnesium sulfate . Usually, however, monitoring and symptomatic treatment is sufficient because it is a self-limiting disease.

As a last resort for severe and progressive diseases, there is the intra-arterial administration of Milrinone, Nimodipine or Epoprostenol and the expansion of the arteries by means of balloon dilatation .

forecast

The reversible cerebral vasoconstriction syndrome usually resolves itself within days and weeks.

The long-term prognosis, however, depends on the complications such as the stroke. Most patients recover from the effects of the stroke within a few weeks, but some remain deficient. About five percent of patients develop life-threatening forms with multiple strokes and extensive brain edema.

The combined mortality is less than one percent. Both the complication rate and mortality could be increased in RCVS after childbirth.

There may be recurring symptoms; how often this is the case is unclear. However, the rate is likely to be quite low as there are few reports of it.

See also

literature

Web links

Individual evidence

  1. ↑ a b c A. Ducros: Reversible cerebral vasoconstriction syndrome. In: Lancet Neurol. 11, 2012, pp. 906-917.