The tropomyosin-receptor kinase fused gene (TFG) has recently been implicated in several distinct hereditary disorders, including the autosomal-recessive form of complicated hereditary spastic paraplegia called SPG57. Previously, three homozygous variants of the TFG gene were reported in five families with SPG57, in which early onset spastic paraplegia, optic atrophy, and peripheral neuropathy were variably identified. Here, we present the first Japanese patient with SPG57, and have added a homozygous p.Ile66Thr variant as the fourth SPG57 genotype.
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http://dx.doi.org/10.1038/s10038-018-0538-4 | DOI Listing |
Cureus
December 2024
Internal Medicine, Cooper University Hospital, Camden, USA.
This case report presents a complex and challenging scenario of recurrent () bacteremia and tricuspid valve endocarditis in a 77-year-old male patient with multiple comorbidities and indwelling medical devices. The patient's medical history was significant for T4 paraplegia, neurogenic bladder requiring a chronic indwelling suprapubic catheter, heart block status post-permanent pacemaker placement, type 2 diabetes mellitus, chronic kidney disease, and chronic sacral wounds. The case highlights the difficulties in managing antibiotic-resistant infections, particularly in patients with implantable devices and chronic wounds.
View Article and Find Full Text PDFAnn Clin Transl Neurol
January 2025
Movement Disorders Program, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Biallelic loss-of-function variants in AP4S1 cause childhood-onset hereditary spastic paraplegia. A recent report suggested that heterozygous AP4S1 variants lead to a syndrome of lower limb spasticity and dysregulation of sphincter function. We critically evaluate this claim against clinical observations in 28 heterozygous carriers of the same AP4S1 variant (NM_007077.
View Article and Find Full Text PDFGenes (Basel)
December 2024
Genomic Medicine Laboratory UILDM, IRCCS Santa Lucia Foundation, 00179 Rome, Italy.
Background/objectives: Artificial intelligence and large language models like ChatGPT and Google's Gemini are promising tools with remarkable potential to assist healthcare professionals. This study explores ChatGPT and Gemini's potential utility in assisting clinicians during the first evaluation of patients with suspected neurogenetic disorders.
Methods: By analyzing the model's performance in identifying relevant clinical features, suggesting differential diagnoses, and providing insights into possible genetic testing, this research seeks to determine whether these AI tools could serve as a valuable adjunct in neurogenetic assessments.
Indian J Pediatr
January 2025
Department of Pediatrics, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba-shi, Chiba, 260-8670, Japan.
Neuroradiology
January 2025
Department of Radiology, The First Affiliated Hospital of Fujian Medical University, No. 20, Chazhong Rd., Taijiang District, Fuzhou, 350005, Fujian, China.
Purpose: Spastic paraplegia type 5 (SPG5) is a rare neurodegenerative disease diagnosed primarily through genetic testing.We identified a specific spinal cord sign on conventional MR imaging to help narrow the scope of genetic screening.
Methods: In 25 patients with SPG5 and 21 healthy controls (HCs), the spinal cord cross sign was evaluated on T2*-weighted imaging.
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