Acute Neurological Manifestations of Porphyrias and its Types: A Systematic- Review.

INTRODUCTION: Acute porphyrias cause life-threatening attacks of neurovisceral non-specific symptoms, so this condition mimics many acute medical and psychiatric diseases. The disease is very misdiagnosed, probably due to its low incidence and non-pathognomonic symptoms, this delays the effective treatment onset. Early diagnosis and treatment highly improve the prognosis and can prevent the development of neuropathic manifestations. METHODS: We assembled a systematic review, following the PRISMA guidelines and using Pubmed as our database. Our aim was to show some peculiarities among patients that present neurological manifestations in acute porphyria attack. We obtained the patients' age, sex, clinical presentation, eurological manifestations and porphyria type of 16 patients. We also evaluated the time between symptoms onset and neurological manifestations. The average age was 28,4 ± 11,1; 50% of patients were male. RESULTS: AIP was the most prevalent porphyria type. The average time between symptoms onset and neurological manifestations was of 9,53 ± 11,6 days. Abdominal pain; nausea and vomiting and psychiatric manifestations were the most common symptoms preceding neurological attacks. Seizures and consciousness disturbance were the most prevalent findings within an attack. We also presenting a case to illustrate how difficult this diagnosis can be.

Pseudarthrosis in anterior cervical discectomy and fusion with a self-locking, stand-alone cage filled with hydroxyapatite: a retrospective study with clinical and radiological outcomes of 98 levels with a minimum 2-year follow-up.

OBJECTIVE: The goal of this study was to evaluate the incidence of pseudarthrosis after the treatment of cervical degenerative disc disease (CDDD) with anterior cervical discectomy and fusion (ACDF) in which self-locking, stand-alone intervertebral cages filled with hydroxyapatite were used. METHODS: The authors performed a retrospective cohort study of 49 patients who underwent 1- to 3-level ACDF with self-locking, stand-alone intervertebral cages without plates, with a minimum 2 years of follow-up. The following data were extracted from radiological and clinical charts: age, sex, time and type of pre- and postoperative signs and symptoms, pain status (visual analog scale [VAS]), functional status (Neck Disability Index [NDI]), history of smoking, bone quality (bone densitometry), and complications. Pseudarthrosis was diagnosed by a blinded neuroradiologist using CT scans. Clinical improvement was assessed using pre- and postoperative comparison of VAS and NDI scores. The Wilcoxon test for paired tests was used to evaluate statistical significance using a p value of < 0.05. RESULTS: Three patients (6%) developed symptomatic pseudarthrosis requiring reoperation, with only 1 patient showing clinical worsening due to pseudarthrosis, while the other 2 with pseudarthrosis had associated disc disease at an adjacent level. The rate of symptomatic pseudarthrosis according to the number of operated levels was 0% for 1 level, 8.7% (2/23 patients) for 2 levels, and 7.7% (1/13 patients) for 3 levels. The total pseudarthrosis rate (including both symptomatic and asymptomatic patients) was 16.4%. Considering the clinical outcomes, there was a significant improvement of 75.6% in neck pain and 95.7% in arm pain, as well as a 64.9% improvement in NDI scores. Complications were observed in 18.4% of patients, with adjacent-level degenerative disease being the most prevalent at 14.3%. CONCLUSIONS: ACDF with self-locking, stand-alone cages filled with a hydroxyapatite graft can be used for the surgical treatment of 1- to 3-level CDDD with clinical and radiological outcomes significantly improved after a minimum 2-year follow-up period. Comparative studies are necessary.