Successful repeated gastric peroral endoscopic myotomy (re-G-POEM) in the treatment of postsurgical gastroparesis.

INTRODUCTION: Gastric peroral endoscopic myotomy (G-POEM) is a promising but still experimental endoscopic method for the treatment of gastroparesis. CASE REPORT: We describe the case of a 31 years old female patient with severe refractory postsurgical gastroparesis preceded by fundoplication done for reflux disease. She has had digestive problems since childhood. In 2018, the first G-POEM was performed but did not lead to any clinical improvement. We proposed to perform a repeated G-POEM given that impedance planimetry measurement showed persistent pylorospasm. The patient agreed and the second G-POEM was performed in February 2021 (the first re-G-POEM in the Czech Republic) with a satisfactory clinical result; a subsequent gastric emptying study also showed a significant improvement. CONCLUSION: This case report shows the feasibility of performing a repeated endoscopic pyloromyotomy in patients with gastroparesis and proven pylorospasm. One can only hypothesize whether the lack of effect of the first procedure was caused by an incomplete myotomy (failure of the procedure) or the need for a double myotomy, which is considered a standard in some centers.

Per-oral endoscopic pyloromyotomy (g-poem) for the treatment of gastroparesis - a pilot single-centre study with mid-term follow-up.

INTRODUCTION: Endoscopic pyloromyotomy (G-POEM) is an emerging therapeutic method for the treatment of gastroparesis (GP). So far, only limited case-series suggesting its effectivity have been published. The aim of our study was to assess the effectivity of G-POEM in patients with refractory GP. METHODS: Consecutive patients with severe and refractory GP were offered the procedure. An abnormal gastric emptying study (GES) was necessary for inclusion. The main outcome was treatment success defined as a decrease of the total GSCI symptom score by at least 40% from baseline at 3, 6, 12 and 24 months. RESULTS: G-POEM was performed in 9 patients (5 women, mean age 56.3): 5 post-surgical, 2 diabetic, 1 idiopathic and 1 combined post-surgical and diabetic. The median follow-up was 23M (range 12-31). All procedures were successfully completed. One patient experienced delayed bleeding from gastric ulceration, which was successfully treated endoscopically; all remaining patients recovered uneventfully. Treatment success was achieved in 8/9 patients (88.9%) at 3, 6 and 12M and in 3/4 (75%) at 24M. The mean GSCI decreased from 3.16 to 0.86 (p=0.008), 0.74 (p=0.008), 1.07 (p=0.008) and 1.31 (p=0.11) at 3, 6, 12 and 24M after the procedure. The Quality of Life Index improved from the baseline value of 77 (range 48102) to 113 (86-138, p=0.03) and 96 (50-124, p=0.4) at 12 and 24M. In patients with treatment success, no recurrences have occurred so far. GES improved/normalized in all the patients. CONCLUSION: G-POEM was effective in 88.9% of patients with refractory GP and the effect seems to be long-lasting.

Perfusion scintigraphy in the assessment of autologous cell therapy in diabetic patients with critical limb ischemia.

Perfusion scintigraphy with technetium-99-methoxy-isobutyl-isonitrile ((99m)Tc-MIBI) is often used for assessing myocardial function but the number of studies concerning lower limb perfusion is limited. The aim of our study was to assess whether (99m)Tc-MIBI was an eligible method for evaluation of the effect of cell therapy on critical limb ischemia (CLI) in diabetic patients. (99m)Tc-MIBI of calf muscles was performed before and 3 months after autologous cell therapy (ACT) in 24 diabetic patients with CLI. Scintigraphic parameters such as rest count and exercising count after a stress test were defined. These parameters and their ratios were compared between treated and untreated (control) limbs and with changes in transcutaneous oxygen pressure (TcPO(2)) that served as a reference method. The effect of ACT was confirmed by a significant increase in TcPO(2) values (p<0.001) at 3 months after ACT. We did not observe any significant changes of scintigraphic parameters both at rest and after stress 3 months after ACT, there were no differences between treated and control limbs and no association with TcPO(2) changes. Results of our study showed no significant contribution of (99m)Tc-MIBI of calf muscles to the assessment of ACT in diabetic patients with CLI over a 3-month follow-up period.

Relationship between ALS and the degree of cognitive impairment, markers of neurodegeneration and predictors for poor outcome. A prospective study.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a common neurodegenerative disease affecting motor neurons and may be associated with impaired cognition. Reliable prognostic factors for ALS patients are still missing. METHODS: We prospectively included 67 patients, 42 women and 25 men, with clinically defined ALS. The disease severity was assessed and the patients underwent SPECT, lumbar puncture with determination of tau, hyperphosporylated tau (p-tau) and beta-amyloid and a detailed neuropsychological assessment using a standardized test battery. In patients who died, a detailed neuropathologic evaluation was performed. RESULTS: The mean survival duration was 26.8 months. The delay between the first signs and confirmation of the diagnosis was 12.75 months. Cognitive impairment did not have an impact on the evolution of the disease. There was no correlation between neuropsychological and SPECT findings. Higher age at onset, more pronounced handicap and elevated beta-amyloid in the CSF were associated with shorter survival times. In brain tissue from nine of the deceased patients with ALS and dementia, all showed signs of comorbidity, six had hallmarks of frontotemporal lobar degeneration (FTLD) and three showed Alzheimer disease pathology. Brain tissues form 11 deceased ALS patients who did not show signs of dementia, had only changes compatible with a diagnosis of motor neuron disease. CONCLUSION: In our prospective study, age, disease severity and CSF beta-amyloid levels taken together were a risk factor suggesting shorter survival times. Dementia is relatively frequent in ALS and may be a consequence of either FTLD or result from co-existing Alzheimer disease.

[Metabolic complications and neurologic manifestations of vitamin B12 deficiency in children of vegetarian mothers]. / Metabolické komplikace a neurologické projevy pri deficitu vitaminu B12 u detí vegetariánských matek.

BACKGROUND: Serious hematological, metabolic and neurological complications owing to the nutritional deficiency of vitamin B12 may occur in infants of mothers on a strict vegetarian diet. METHODS AND RESULTS: The mother of the first child was a strict vegetarian. She had an elevated urinary methylmalonic acid level and a low concentration of serum vitamin B12. Her 13-month-old daughter was exclusively breast-fed until the age of 9 month and then she was fed only vegetables. Physical examination revealed psychomotoric retardation, apathy, muscular hypotonia, abnormal movements and failure to thrive. Laboratory analysis showed a megaloblastic anaemia, a low level of vitamin B12 and methylmalonic aciduria. MRI of the brain revealed diffuse frontotemporoparietal atrophy and retardation of myelination. After treatment with vitamin B12 supplements, abnormal movements disappeared and development improved, but a mild generalised hypotonia continued. A cranial MRI 9 months after treatment still showed signs of retardation of myelination. The second patient, an 8 month-old male, son of a strict vegetarian mother too, was referred for investigation of psychomotoric retardation, hypotonia, dyskinesia, failure to thrive and microcephaly. He was breast-fed and from 6 month of age he had also received fruit juices. Laboratory analysis revealed megaloblastic anaemia, high methylmalonic aciduria and homocystinuria. The patient's and his mother's serum level of vitamin B12 were low. After treatment with vitamin B12 supplements, biochemical and metabolic markers of disease were normal but there continued a generalised hypotonia, microcephaly and language delay. CONCLUSION: Our observations emphasize the health complications of nutritional cobalamine deficiency and a requirement of clinical, biochemical and metabolic monitoring in infants within strict vegetarian families.

[Acute osteomyelitis in children--modification of scintigraphic examination of the skeleton]. / Akutní osteomyelitida u detí--modifikace scintigrafického vysetrení skeletu.

The authors review diagnostic possibilities of acute osteomyelitis in children. They emphasize above all isotope examinations as the method of choice. In the subsequent part they focus attention on possible differentiation of acute osteomyelitis from inflammations of soft tissues-phlegmons. To this end they used a modified method of Israel published in 1987, based on a chronologically different uptake of 99mTc-MDP in normal bone and bone affected with osteomyelitis. According to this method it is possible to differentiate with a 90% accuracy acute osteomyelitis from phlegmons.