Pischik E1, Kauppinen R2
1Department of Medicine, Division of Endocrinology, University Central Hospital of Helsinki, Biomedicum Helsinki, Finland; Department of Neurology, Neuromuscular Unit, City Hospital #2, Pavlov State Medical University, Saint Petersburg, Russia 2Department of Medicine, Division of Endocrinology, University Central Hospital of Helsinki, Biomedicum Helsinki, Finland
AIP exist. Scales designed for other diseases, including those accompanied by peripheral neuropathy, are not suitable for acute porphyrias since many specific features and their combinations are characteristic for acute porphyrias. Clinical manifestations and outcome of an acute attack in 12 Russian AIP patients with severe neurological manifestations during an acute attack were used as a basis for developing a scale by factor analysis. Assessment of muscle strength was performed according to Medical Research Council scale (MRC) from 0 to 5 grades in six muscle groups from both sides (MRC score sum in healthy subject = 60). Intensity of pain was assessed using linear Visual Analogue Scale (VAS, 0 to 10 cm). According to factor analysis, muscle weakness (MRC score sum 25.5±20.2 corresponding to mean 3.6 scores, range 0-6), prolonged mechanical ventilation (from 21.3±20.1 days, mean 2.1 scores, range 0-6), bulbar palsy (mean 2.1 scores, range 0/6), impaired consciousness (mean 1.4 scores, range 0-6), hyponatremia (mean 123.5±9.2 mmol/L, mean 1.9 scores, range 0-6) and arrhythmia (mean scores 0.3, range 0-3) suggested a poor prognosis (max total scores = 33). Other signs and symptoms, such as abdominal pain, tachycardia, hypertension, vomiting, constipation and sensory loss did not affect the prognosis and epileptic seizures correlated with a prognosis only slightly. Thus, they were excluded from the scale (scaled 0). In conclusion, the total score predicted the short and long term-outcome (I:6 months or residual signs; III:>25 scores, critical condition, p=0.0004) and attacks could be classified as mild (=0 scores), moderate (1-4 scores), severe (5-25 scores) or critical (26 -33 scores). In mild attacks, measuring the pulse and following intensity of pain may indicate the activity of the disease. A form to follow-up a patient's clinical condition for evaluation of scoring will be presented.