Case report: Myelitis and ganglionitis, an atypical presentation of Hansen's disease.

Hansen's disease, or leprosy, is a disease characterized by dermatological and neurological disorders. A neural form also exists, in which peripheral neuropathy occurs in the absence of skin lesions. However, cases of leprosy that involve the central nervous system and proximal nerves are rare in the literature. We describe the case of an oligosymptomatic patient diagnosed with the neural form of leprosy with involvement of peripheral nerves, dorsal root ganglion, and cervical spinal cord in an atypical presentation of the disease. Through complementary examinations and nerve biopsies, the bacillus was identified, and treatment was subsequently initiated. This case highlights the importance of investigating the suspicion of leprosy, even in cases with atypical manifestations, as early diagnosis and treatment can reduce neurological damage and deformities.

Deep Vein Thrombosis in the Setting of Neurofibromatosis Type 1: Case Report

Neurofibromatosis type 1 (NF1) is a genetic syndrome which typically presents with neurological manifestations. Some of the patients may also present with vasculopathies, among which arterial aneurysms and stenosis are the most common. Deep vein thrombosis (DVT) has rarely been described, and, to the best of our knowledge, the present is the first report of DVT due to venous compression by a neurofibroma in the setting of NF1. This is the case of a 23-year-old male with NF1 who experienced DVT due to compression of the left posterior tibial veins by a large tumor arising from the tibial nerve. The DVT was acutely treated with enoxaparin and then with rivaroxaban. Two months after the diagnosis, Doppler ultrasonography showed partial recanalization and persistence of the DVT. The patient was then referred to neurosurgery for surgical resection of the tumor. There were no complications during the procedure, and the patient did not present postoperative neurological deficits. The final histopathological diagnosis was of a benign neurofibroma. After one year of follow-up with vascular surgery, the patient presented no more episodes of DVT. In case there is a tumor compressing the deep vessels of the leg and promoting DVT, surgical resection with microsurgical techniques may be curative.

Surgical Management of Symptomatic Lumbar, Sacral, and Lumbosacral Plexus Tumors: a Peripheral Nerve Unit Experience.

OBJECTIVE: Data concerning the surgical treatment of lumbosacral plexus tumors (LSPTs) is scarce. This study aims to present our experience with a series of 19 patients surgically treated for symptomatic LSPTs at our institution. METHODS: This is a retrospective study of 19 patients surgically treated for symptomatic LSPTs from 2011 to 2019. Clinical data were retrieved from medical records and consisted of age, gender, clinical presentation, location of the lesion, surgical approach, final histopathologic diagnosis, follow-up time, outcomes, and complications. RESULTS: Nineteen surgical procedures were conducted. Thirteen patients were female and six, male. The median age of patients was 45 years (range 20 to 63 years). No patients harbored genetic syndromes. Surgical treatment appears to be correlated to the reduction of pain in patients with peripheral nerve sheath tumors (PNSTs), as assessed by visual analog scale (VAS). Sixteen patients did not present with new-onset deficits during follow-up (84.2%), two of whom recovered from their preoperative deficit. Four patients presented with postoperative weakness. The histopathological diagnoses were 11 schwannomas, four neurofibromas, three metastases, and one lymphoma. CONCLUSIONS: LSPTs are rare. When surgical treatment is indicated, it usually requires multidisciplinary management. Surgery appears to be effective concerning the reduction of pain in PNSTs and may also recover neurological deficits. Iatrogenic neurological deficits are an evident risk, such that intraoperative multimodal monitoring should always be performed if available. In lesions involving the sacral plexus, we found it to be indispensable.

Nondiscogenic Sciatica: What Clinical Examination and Imaging Can Tell Us?

OBJECTIVE: Lesions affecting the sciatic nerve (SN) can mimic lumbar radiculopathy. In patients presenting with sciatica, approximately 10% have a nondiscogenic etiology. Through neurological examination and imaging, it may be possible to confirm nondiscogenic sciatica (NDS). This study aims to present a series of 6 patients with infragluteal NDS, highlighting clinical and imaging aspects that may suggest this diagnosis. METHODS: This is a retrospective study of 6 patients treated for NDS from 2010 to 2018. The mean and median ages were 41.2 and 38.5 years, respectively, with all patients female. RESULTS: All patients presented with sciatic pain, tenderness to deep infragluteal palpation, and a positive Tinel's sign related to the SN. Four patients were referred for surgical treatment, whereas 1 underwent pharmacological therapy and 1 underwent incisional biopsy and radiotherapy. In our series, 6 different causes for NDS were diagnosed: 1 nontumorous cause: extrauterine endometriosis and 5 tumors: metastasis from rectal adenocarcinoma, low-grade sarcoma, schwannoma, high-grade sarcoma, and myxoma. CONCLUSIONS: Differentiating between discogenic and NDS can be challenging for clinicians. When patients present with sciatic pain, a Tinel's sign related to the SN elicited at the deep infragluteal region, tenderness to deep infragluteal palpation, occasionally with an SN motor deficit and imaging findings of the lumbar spine that do not justify a discogenic source, the cause should be considered nondiscogenic and they should be scheduled for magnetic resonance imaging of the gluteal and pelvic region.

Extradural lumbar nerve root cavernous hemangioma: case report and successful surgical resection / Hemangioma cavernoso extradural de raiz nervosa lombar: relato de caso e ressecção cirúrgica bem-sucedida

Extradural lumbar spinal canal cavernous hemangiomas (or cavernomas) are rare lesions that can induce intense back pain and neurological deficit. We present a case report of a patient with a pure radicular lombar extradural cavernoma resembling a benign neurological tumor in imaging exams and a successful surgical resection.

Os hemangiomas cavernosos do canal vertebral lombar extradural (ou cavernomas) são lesões raras que podem induzir dor intensa no dorso e déficit neurológico. Apresentamos um relato de caso de um paciente com um cavernoma extradural lombar radicular puro assemelhando-se a um tumor neurológico benigno em exames de imagem e uma ressecção cirúrgica bem-sucedida.

Increased extracellular matrix deposition during chondrogenic differentiation of dental pulp stem cells from individuals with neurofibromatosis type 1: an in vitro 2D and 3D study.

BACKGROUND: Neurofibromatosis 1 (NF1) presents a wide range of clinical manifestations, including bone alterations. Studies that seek to understand cellular and molecular mechanisms underlying NF1 orthopedic problems are of great importance to better understand the pathogenesis and the development of new therapies. Dental pulp stem cells (DPSCs) are being used as an in vitro model for several diseases and appear as a suitable model for NF1. The aim of this study was to evaluate in vitro chondrogenic differentiation of DPSCs from individuals with NF1 using two-dimensional (2D) and three-dimensional (3D) cultures. RESULTS: To fulfill the criteria of the International Society for Cellular Therapy, DPSCs were characterized by surface antigen expression and by their multipotentiality, being induced to differentiate towards adipogenic, osteogenic, and chondrogenic lineages in 2D cultures. Both DPSCs from individuals with NF1 (NF1 DPSCs) and control cultures were positive for CD90, CD105, CD146 and negative for CD13, CD14, CD45 and CD271, and successfully differentiated after the protocols. Chondrogenic differentiation was evaluated in 2D and in 3D (pellet) cultures, which were further evaluated by optical microscopy and transmission electron microscopy (TEM). 2D cultures showed greater extracellular matrix deposition in NF1 DPSCs comparing with controls during chondrogenic differentiation. In semithin sections, control pellets hadhomogenous-sized intra and extracelullar matrix vesicles, whereas NF1 cultures had matrix vesicles of different sizes. TEM analysis showed higher amount of collagen fibers in NF1 cultures compared with control cultures. CONCLUSION: NF1 DPSCs presented increased extracellular matrix deposition during chondrogenic differentiation, which could be related to skeletal changes in individuals with NF1.

Traumatic arteriovenous fistula associated to pseudoaneurysm of the superficial temporal artery in a child: case report and literature review / Fístula arteriovenosa traumática associada a pseudoaneurisma da artéria temporal superficial em uma criança: relato de caso e revisão da literatura

As fístulas arteriovenosas (FAVs) e os pseudoaneurismas traumáticos extracranianos são malformações incomuns e, em sua maioria, estão associadas a traumatismo craniano fechado com lesão contusa de forte intensidade. O diagnóstico em geral é clínico, porém o exame de escolha para o diagnóstico definitivo é a angiografia. Nos casos em que a lesão é pequena, é possível abordá-la com embolização via endovascular com sucesso. A excisão cirúrgica, no entanto, ainda é o método de escolha para o tratamento. É relatado um caso de um paciente do sexo masculino, com 9 anos de idade, diagnosticado com FAV, acometendo o ramo frontal da artéria temporal superficial, secundária a trauma craniano contundente ocorrido três anos antes do diagnóstico.

The arteriovenous fistulae (AVFs) and the extracranial traumatic pseudoaneurysms are uncommon malformations and in the majority of the cases are associated to closed head trauma with high intensity blunt lesion. The diagnosis is generally clinical, though the exam of choice for definitive diagnosis is an angiography. In minor lesion cases it's possible to successfully approach it with endovas-cular embolization. The surgical excision though, is the method of choice for the treatment. Here is reported a case of a 9-year-old male patient, diagnosed with AVF involving the frontal branch of the superficial temporal artery, secondary to blunt head trauma occurred three years before diagnosis.