IgM paraprotein and anti-MAG sensory polyneuropathy associated with Waldenstrom's macroglobulinaemia and medullary carcinoma of the thyroid.

A previously fit and well 76-year-old man, presented with distal lower limb sensory symptoms suggestive of peripheral sensory neuropathy, associated with positive anti-MAG antibodies (myelin associated glycoprotein) and IgM paraprotein. Bone marrow biopsy showed lymphoplasmocytoid lymphoma (Waldenstrom's macroglobulinaemia, WM), consequently positive for MYD88 mutation. He subsequently developed medullary carcinoma of the thyroid, most likely secondary to WM. He underwent a successful total thyroidectomy and four treatment doses of rituximab, which proved beneficial. He is currently stable and under multidisciplinary monitoring. His sensory symptoms have improved following rituximab treatment and his WM is under control.

Neuromyelitis optica spectrum disorder (NMOSD) presenting as acute transverse myelitis with positive aquaporin 4 antibodies.

An 80-year-old, previously healthy patient presents with acute transverse myelitis with sensory level at T8. The MRI scan of the spinal cord showed longitudinal extensive transverse myelitis, and she tested positive for aquaporin 4 antibodies in serum. She received treatment with intravenous and oral steroids, with no improvement and then underwent plasma exchange. She was then started on azathioprine for prevention of relapses, while continuing physiotherapy and occupational therapy. Eventually, she was transferred to a specialised spinal cord centre for long-term rehabilitation.

Bilateral, vertical supranuclear gaze palsy following unilateral midbrain infarct.

A 60-year-old man recently admitted for bipedal oedema, endocarditis and a persistently positive COVID-19 swab with a history of anticoagulation on rivaroxaban for atrial fibrillation, transitional cell carcinoma, cerebral amyloid angiopathy, diabetes and hypertension presented with sudden onset diplopia and vertical gaze palsy. Vestibulo-ocular reflex was preserved. Simultaneously, he developed a scotoma and sudden visual loss, and was found to have a right branch retinal artery occlusion. MRI head demonstrated a unilateral midbrain infarct. This case demonstrates a rare unilateral cause of bilateral supranuclear palsy which spares the posterior commisure. The case also raises a question about the contribution of COVID-19 to the procoagulant status of the patient which already includes atrial fibrillation and endocarditis, and presents a complex treatment dilemma regarding anticoagulation.

Waldenstrom-associated anti-MAG paraprotein polyneuropathy with neurogenic tremor.

A 71-year-old female patient presented with a 14-year history of slowly progressive distal limb numbness, paraesthesia and reduced vibration perception, ataxic gait and intentional tremor. Examination revealed with a length-dependent sensory neuropathy. Nerve conduction studies showed a chronic sensorimotor inflammatory demyelinating polyneuropathy. Intravenous immunoglobulin treatment (on two occasions) proved ineffective. Serum electrophoresis showed increased monoclonal IgM with kappa light chains. Anti-myelin-associated glycoprotein (MAG) levels were extremely elevated, >70 000 BTU. Bone marrow biopsy revealed 15%-20% small B cells and positive MYD88 mutation, indicative of Waldenstrom macroglobulinaemia. A diagnosis of Waldenstrom-associated anti-MAG paraprotein neuropathy with intentional (neurogenic) tremor was made. Repeat nerve conduction study showed a severe sensory demyelinating neuropathy with no axonal lesion. Treatment with rituximab was given for 1 month with minimal improvement. Repeat anti-MAG levels dropped to 53 670 BTU, with minimal clinical improvement.

Developing new SrI2 and ß-D-fructopyranose-based metal-organic frameworks with nonlinear optical properties.

In the context of personalized medicine, there is a growing interest in materials bearing at the same time diagnostic and therapy functions. This article reports a cheap and easily reproducible procedure to obtain materials with a high potential for these applications. Three new strontium iodide-fructose-based metal-organic frameworks with formulae [Sr(C6H12O6)2]I2, [Sr2(C6H12O6)3(H2O)3]I4·0.5H2O and [Sr(C6H12O6)(H2O)3I]I differing in stoichiometry, symmetry and crystal packing, were obtained and characterized by X-ray diffraction. Bulk quantum simulations show that both the ions and the sugar are crucial in determining the predicted nonlinear response; also, the relative arrangement of various functional groups in the unit cell plays a role in the computed optical properties. Small fragments of the three compounds were selected for in vacuo calculations, proving that the reduced dimensions of the particles have a great influence on the nonlinear optical response. Despite the similar chemical composition of the three compounds, second harmonic generation measurements and in crystal and in vacuo theoretical calculations agree that one of the compounds is a much more efficient second harmonic emitter than the other two, and is thus a suitable candidate for bio-sensor applications.

Reversible and unilateral corticospinal tract disease secondary to autoimmune free-T3-thyrotoxicosis.

68-year-old female patient with no significant medical history presents with a 3-month history of progressive neurological symptoms, which began with left eye ptosis, blurred vision and non-painful jaw discomfort, followed by left spastic weakness and hyper-reflexia with positive Babinski and Hoffman signs. An elevated T3 level, a positive peroxidase and an antigraves antibody level led to an ultrasound, which confirmed a sub acute-chronic autoimmune thyroiditis. A nerve conduction studies/electromyogram showed normal motor and sensory velocity conduction with a small amplitude compound motor action potential, indicative of likely axonal damage. Following treatment with carbimazole, the neurological symptoms greatly improved. The authors concluded that the left pyramidal syndrome was secondary to autoimmune free T3-thyrotoxicosis.

An elusive ciliopathy: Joubert syndrome.

The police brought a 65-year-old female patient to the EADU after being found 'roaming the streets' in an apparent state of confusion. This was her third admission under the same circumstances during the last 3 years. Neurological examination revealed (1) cognitive impairment, (2) oculomotor apraxia, (3) abnormal cancellation of vestibular ocular reflex, (4) mild ataxia and (5) mild hypotonia. Renal function was abnormal and liver function was normal. No retinal disturbance was found. The head CT on admission was normal for stroke and the lumbar puncture was negative for encephalitis. Her brain MRI showed 'molar tooth sign', suggestive of Joubert syndrome, which was confirmed by genetic testing showing anomalous NPHP1 gene.

Progressive bilateral ophthalmoparesis--a case of simultaneous autoimmunity: balancing Graves' ophthalmoparesis and ocular myasthenia.

A 44-year-old woman with no medical history presented with a 1-year history of horizontal diplopia, bilateral exophthalmos and progressive asymmetrical ophthalmoparesis, with no pupillary dysfunction or ptosis. Within 3 months of her initial presentation, she noticed paresis of right eye abduction, followed after 1 month with paresis of left eye abduction. Initial investigations revealed positive antiperoxidase antibodies for Graves' disease and positive AChR for myasthenia gravis. MRI of the brain showed increased intensity in bilateral inferior rectus muscles and CT of the chest showed thymic hyperplasia. Treatment with carbimazole and pyridostigmine was started, with complete resolution after 1 month.

A deceitful stroke, a hidden tumour.

A 41-year-old woman with malignant left middle cerebral artery infarction underwent an immediate decompressive craniectomy, obtaining good recovery, both clinically and radiologically. However, approximately 6 months after a successful discharge, the patient returned with gradual memory deterioration, dysarthria and progressively worsening headaches. Subsequent CT and MRI of the brain revealed a solid malignancy located in the same region as the extensive infarction. Following this finding, she underwent surgical debulking of the tumour and follow-up radiotherapy, with good progression of symptoms and overall good outcome.

A model study on the absorbed dose of radiation following respiratory intake of 238U3O8 aerosols.

Aerosols of depleted uranium oxides, formed upon high-energy impact of shells on hard targets during military operations, are able to disperse, reach the alveolar region of the lungs and be absorbed and distributed throughout various parts of the body. The absorbed particles are subjected to clearance in the upper respiratory tract, distribution to other body districts, dissolution and excretion. While the soluble forms of uranium are known to deliver a small dose of radiation to the body due to their homogeneous distribution and the low specific activity of (238)U, ceramic particles exhibit a low dissolution rate and irradiate a limited volume of tissue for a long time with alpha particles with an energy of 4.267 MeV. The extent of the irradiated tissues depends on the radius of the particles and the total intake of uranium oxides. For the measured intake of U3O8 of a war veteran (15.51 µg) the number of particles ranges from 5.56×10(4) to 6.95×10(6) for sizes of 0.4-2.0 µm. Modelling the distribution of the particles between two compartments of the body, the averaged dose absorbed in 20 y by tissues surrounding the particles and within the range of the alpha particles varies from 6.8 mGy to 0.85 Gy for lungs and 8.1 mGy to 1.0 Gy for the lymph nodes, respectively. Correspondingly, due to the clearance and redistribution, the mass irradiated by 2.0-µm particles falls in 20 y from 6.06 mg to 0.94 µg in the lungs and grows from 0 to 1.0 mg in the lymph nodes. The estimated rate of formation of hydroxyl radicals upon radiolysis of water in the lungs and lymph nodes is 5.17×10(4) d(-1) per cell after 1 y.

Desequilibrio esfuerzo-recompensa y estado de malestar mental en trabajadores de Servicios de Salud en Chile / Effort-reward unbalance and mental uneasiness state in health services workers in Chile

El presente estudio es de tipo exploratorio y aborda la asociación entre las dimensiones del modelo de estrés laboral (Effort Reward Imbalance, DER), y el modelo de salud mental (General Health Questionnaire, GHQ-28). Se basa en entrevistas y encuestas realizadas a 68 trabajadores del sector salud en 3 lugares de Santiago, Chile (un hospital privado, un hospital público y un consultorio de atención primaria). Los participantes incluyen auxiliares de enfermería, técnicos paramédicos y personal administrativo de varios departamentos. Se encontró asociaciones positivas entre el mal estado de salud mental y la presencia de estrés laboral.

This study explored the association between dimensions of a job-stress model, Effort-Reward Imbalance (ERI), and a mental health model, the General Health Questionnaire (GHQ-28). The exploratory study was based on interviews and self-response questionnaires given to 68 health care workers at three worksites in Santiago, Chile (a large public hospital, a large private hospital, and a primary care clinic). The participants included nursing assistants, technicians, and administrative staff from various departments. Positive associations were found between bad mental health and job stress.

Regioselectivity Patterns Featured by Formaldehyde in the Electrophilic Addition to gem-Difluoro and gem-Dichloroallyl Systems. An ab Initio Theoretical Study.

The addition reactions of formaldehyde with (1,1-difluoroallyl)lithium and (1,1-dichloroallyl)lithium and -potassium (and corresponding free anions) have been investigated at the HF and MP2 levels of theory. The two competing pathways leading from the initial electrostatic sigma-complexes to the alpha and gamma addition products have been studied. In (1,1-difluoroallyl)lithium, (1,1-dichloroallyl)potassium, and both free anions the alpha-pathway is sharply preferred. In contrast, for (1,1-dichloroallyl)lithium the difference between the two activation energies is smaller and in favor of the gamma-pathway: as a consequence, on the basis of the present calculations, it is expected that in this case varying substituents on the electrophilic carbon could modulate the regioselective preferences. All results appear to be consistent with available experimental evidences. A balance between an exothermicity factor and the different destabilization undergone by the various reactants in the transition structures offers a qualitative rationalization of the opposite regioselective inclinations of (1,1-dichloroallyl)lithium with respect to the other systems.