PMC9662787_01
The evolution of clinical symptoms and urinary porphyrins through the follow up. The vertical red band labels the acute abdominal pain period supposedly corresponding the acute porphyria attack, while the light green is related to the stationary neurorehabilitation. The numbers within the grey circles represent the key observation periods corresponding the main evolutionary/intervention steps, as following (M month, the digit following M represents the number of months before or after the gastric bypass procedure): (1) [M-48 until 01.2018 (M0)] is the retrospective period; (2) [from M0 to M11 (01.2019)] corresponds to the period during which the main and most severe symptoms as well as therapeutic interventions in hospitals occurred; (3) [from M11 until the end of the follow up at M21 (12.2019)] represents the post-stationary evolution. Urinary porphyrins concentrations are labelled as following: turquoise blue dots for ALA, dark blue rhombs for PBG and violet squares for total porphyrins. Main clinical symptoms evolution over the observation period. Ordinate values are arbitrary units corresponding to a clinical estimate of symptom severity. The main symptoms were grouped in three categories represented by three different color codes: abdominal pain in orange, peripheral neuropathic signs in yellow and psychiatric symptoms in grey. The functional performance and main interventions are shown by illustrative cartoons: the stomach for bariatric surgery, the bed for the period when the patient was bed-ridden, the wheelchair and walking stick and free walking showing the improving patterns of mobility. (1) burn-out 2014 (grey pyramid); (2) acute abdominal pain with maximal intensity during the period considered to correspond to the porphyria attack, with small peaks occurring at surgical interventions (small orange spines above the baseline). The pain intensity decreased as rehabilitation and other treatments were initiated, and stabilized at very low level although 3 repetitive peaks took place at M7-M8. Of notice, two isolated peaks were recorded during appendagitis exacerbation (with * above) at M4 and M11. Neuropathic symptoms started later than abdominal pain as characteristic for porphyria. They were at their maximum from the beginning, but progressively decreased during neurorehabilitation program parallel to the overall clinical state of the patient. They finally dropped to a low, but persistent steady-state level; (3) during this post-stationary period, both abdominal pain and neuropathic symptoms remained low (except two exacerbations of abdominal pain at M16). Psychiatric symptoms including sleep disorders, fatigue, anxiety and depression reappeared, but progressively improved with appropriate medication.
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F1
An intensive multidisciplinary neurorehabilitation regime and optimal symptom treatments allowed the patient to progressively gain her mobility back, with the improvement of her muscle strength, pain, somatosensory complaints, dysphagia, and dysgeusia (Figure 1).