A Rare Presentation of Non-systemic Vasculitic Neuropathy Mimicking Guillain-Barré Syndrome: A Case Report.

Vasculitic neuropathy typically presents as a painful, asymmetrical sensory-motor polyneuropathy, more commonly demonstrating a mononeuritis multiplex. We present the case of a 63-year-old woman who experienced acute-onset flaccid weakness in all four limbs following an episode of diarrhea. Guillain-Barré syndrome (GBS) was considered, which supported acute motor axonal neuropathy (AMAN) in the nerve conduction study (NCS). On the second day of treatment with intravenous immunoglobulin (IVIG), a vasculitic-type rash appeared along with limb pain. Furthermore, the asymmetrical sensory and motor weakness did not respond well to the treatment. A positive skin biopsy, however, with a negative nerve biopsy combined with repeat NCSs demonstrating mononeuritis multiplex, confirmed the diagnosis of non-systemic vasculitic neuropathy (NSVN) based upon Brighton Case Collaboration type 3. This presentation underlines the significance of considering vasculitic neuropathy as a potential diagnosis and highlights the importance of an accurate diagnosis, as this condition can be effectively treated.

Cultural adaptation and validation of the Sinhala version of the Frail Non-disabled tool (FiND).

BACKGROUND: Frailty, a common geriatric syndrome of vulnerability, is associated with a decline in health and function. The most problematic expression of population ageing is associated with weakness, slowing, decreased energy, lower activity and when severe, unintended weight loss. Frailty is not consciously identified in clinical practice and is not widely studied in Sri Lanka. A validated tool for screening frailty in a busy clinical setting is therefore much needed. This study was done as a part of validating the Sinhala version of the Frail Non-Disabled (S-FiND) tool. METHODS: The FiND tool was translated from English to Sinhala by two translators, blinded to each other. They were combined and translated back to the original language by two separate translators. After verifying the content validity, unambiguity and clarity of items in a focused group discussion, the pre-final version was piloted among 30 volunteers. After assessing the psychometric properties of the pre-final version, the final version was tested among 100 adults older than 65 years from the Colombo South Teaching Hospital. The tool was compared with Fried's frailty phenotype taken as the gold standard. RESULTS: Data were analysed for the agreement with the reference standard, the Fried Phenotype. The mean (SD) age of subjects was 73.9 (7.8) years. The overall time taken to fill out the questionnaire was 2 min. The agreement (Kappa) between the S-FiND questionnaire and the Fried phenotype was 0.7 (P < 001). The sensitivity and specificity of FiND in detecting frailty were 92% and 74%, respectively. The agreements (Kappa) between the individual items of S-Find: involuntary loss of weight/ more than 4.5 kg over one year, the feeling of effort/ not getting going and level of physical activity, with the Fried phenotype, were 0.28 (p = 0.001), 0.06 (p = 0.045) and 0.339 (p < 0.001). respectively. When subjects were categorized frail and robust based on FiND, frail subjects reported a higher incidence of falls (50%) during the previous 12 months, compared to those robust (13%) (p < 0.001 for Chi stat). CONCLUSION: The S-FiND is a reliable, valid and well-received tool that can be used in detecting the frailty of non-disabled Sinhala-speaking older adults.

Case report of scrub typhus with bilateral acute sensorineural hearing loss and cerebral salt-wasting disease.

Scrub typhus is a re-emerging and endemic disease in the Asia Pacific region caused by Orientia tsutsugamushi. We present a 65-year-old male from Sri Lanka who presented with fever, bilateral acute sensorineural hearing loss, and confusion. On examination, he was dehydrated. Significant orthostatic hypotension and an eschar were noted. Investigations revealed hyponatraemia with elevated urine sodium, reduced serum osmolality, and normal urine osmolality suggestive of cerebral salt wasting. After initial hydration with 0.9% NaCl, hyponatraemia was corrected with 3% NaCl. Oral doxycycline was prescribed, and he showed dramatic clinical improvement. A diagnosis of typhus must be considered in a patient presenting with a febrile illness and acute hearing loss. Cerebral salt-wasting disease should be considered in a patient with typhus who develops hyponatraemia with dehydration. Furthermore, acute sensorineural hearing loss in both ears is an important manifestation of the disease.

Tetracoordinate Co(II) complexes with semi-coordination as stable single-ion magnets for deposition on graphene.

We present a theoretical and experimental study of two tetracoordinate Co(II)-based complexes with semi-coordination interactions, i.e., non-covalent interactions involving the central atom. We argue that such interactions enhance the thermal and structural stability of the compounds, making them appropriate for deposition on substrates, as demonstrated by their successful deposition on graphene. DC magnetometry and high-frequency electron spin resonance (HF-ESR) experiments revealed an axial magnetic anisotropy and weak intermolecular antiferromagnetic coupling in both compounds, supported by theoretical predictions from complete active space self-consistent field calculations complemented by N-electron valence state second-order perturbation theory (CASSCF-NEVPT2), and broken-symmetry density functional theory (BS-DFT). AC magnetometry demonstrated that the compounds are field-induced single-ion magnets (SIMs) at applied static magnetic fields, with slow relaxation of magnetization governed by a combination of quantum tunneling, Orbach, and direct relaxation mechanisms. The structural stability under ambient conditions and after deposition was confirmed by X-ray photoelectron spectroscopy (XPS) and Raman spectroscopy. Theoretical modeling by DFT of different configurations of these systems on graphene revealed n-type doping of graphene originating from electron transfer from the deposited molecules, confirmed by electrical transport measurements and Raman spectroscopy.

Diabetic Striatopathy: A Case Report of a Patient With Poor Glycaemic Control and Abnormal Movements.

Diabetic striatopathy (DS) is a condition occurring in individuals with type II diabetes mellitus (T2DM) where there are abnormal (usually single-sided) bodily movements (hemiballismus-hemichorea (HBHC)). DS involves the interaction between diabetes leading to damage to areas such as the striatum with the development of a noticeable hyperkinetic movement disorder. Here, we present a case of a 72-year-old man with T2DM, ischaemic heart disease, and dyslipidaemia, who presented with involuntary movements of the bilateral upper limbs (the left side more affected than the right) for three weeks along with progressively worsening subtle involuntary movements of the mouth and tongue, with intact speech, swallowing, and gait. The neurological examination revealed high-amplitude intermittent, sudden onset involuntary movements of the bilateral upper limbs, primarily affecting the left side. Based on clinical findings, which were supported by imaging studies, a diagnosis of diabetic striatopathy was made. His presentation was beyond the classical presentation of unilateral involvement seen in HBHC, but with early identification and strict glycemic control, satisfactory improvement of his clinical status was achieved.

Phenotypic characterization of recessive gene knockout rat models of Parkinson's disease.

Recessively inherited loss-of-function mutations in the PTEN-induced putative kinase 1(Pink1), DJ-1 (Park7) and Parkin (Park2) genes are linked to familial cases of early-onset Parkinson's disease (PD). As part of its strategy to provide more tools for the research community, The Michael J. Fox Foundation for Parkinson's Research (MJFF) funded the generation of novel rat models with targeted disruption ofPink1, DJ-1 or Parkin genes and determined if the loss of these proteins would result in a progressive PD-like phenotype. Pathological, neurochemical and behavioral outcome measures were collected at 4, 6 and 8months of age in homozygous KO rats and compared to wild-type (WT) rats. Both Pink1 and DJ-1 KO rats showed progressive nigral neurodegeneration with about 50% dopaminergic cell loss observed at 8 months of age. ThePink1 KO and DJ-1 KO rats also showed a two to three fold increase in striatal dopamine and serotonin content at 8 months of age. Both Pink1 KO and DJ-1 KO rats exhibited significant motor deficits starting at 4months of age. However, Parkin KO rats displayed normal behaviors with no neurochemical or pathological changes. These results demonstrate that inactivation of the Pink1 or DJ-1 genes in the rat produces progressive neurodegeneration and early behavioral deficits, suggesting that these recessive genes may be essential for the survival of dopaminergic neurons in the substantia nigra (SN). These MJFF-generated novel rat models will assist the research community to elucidate the mechanisms by which these recessive genes produce PD pathology and potentially aid in therapeutic development.

A strategy for the generation, characterization and distribution of animal models by The Michael J. Fox Foundation for Parkinson's Research.

Progress in Parkinson's disease (PD) research and therapeutic development is hindered by many challenges, including a need for robust preclinical animal models. Limited availability of these tools is due to technical hurdles, patent issues, licensing restrictions and the high costs associated with generating and distributing these animal models. Furthermore, the lack of standardization of phenotypic characterization and use of varying methodologies has made it difficult to compare outcome measures across laboratories. In response, The Michael J. Fox Foundation for Parkinson's Research (MJFF) is directly sponsoring the generation, characterization and distribution of preclinical rodent models, enabling increased access to these crucial tools in order to accelerate PD research. To date, MJFF has initiated and funded the generation of 30 different models, which include transgenic or knockout models of PD-relevant genes such as Park1 (also known as Park4 and SNCA), Park8 (LRRK2), Park7 (DJ-1), Park6 (PINK1), Park2 (Parkin), VPS35, EiF4G1 and GBA. The phenotypic characterization of these animals is performed in a uniform and streamlined manner at independent contract research organizations. Finally, MJFF created a central repository at The Jackson Laboratory (JAX) that houses both non-MJFF and MJFF-generated preclinical animal models. Funding from MJFF, which subsidizes the costs involved in transfer, rederivation and colony expansion, has directly resulted in over 2500 rodents being distributed to the PD community for research use.