Adult T-cell leukemia-lymphoma complicated by Takotsubo cardiomyopathy and HTLV-1-associated myelopathy after treatment with the anti-CCR4 antibody mogamulizumab.

Adult T-cell leukemia-lymphoma (ATL) caused by human T-lymphotropic virus type 1 (HTLV-1) is generally associated with poor prognosis. The anti-CCR4 antibody mogamulizumab is one of the options for refractory or relapsed ATL. Mogamulizumab is intravenously administered as a single agent at a dose of 1.0 mg/kg once a week for 8 weeks. The overall response rate is reported to be 50%. Reported common adverse events after administration of mogamulizumab are leukocytopenia, infusion reaction, and skin rash. Although rare, mogamulizumab-associated cardiomyopathy has also been reported. HTLV-1 is also associated with a number of diseases, such as HTLV-1-associated myelopathy (HAM). Approximately 0.25% of infected individuals are estimated to develop HAM. HAM is an inflammatory disease of the central nervous system, which is characterized by slowly progressive spastic paresis of bilateral lower limbs and bladder-rectal disorder. Coexistence of ATL and HAM has rarely been reported. Here we present the case of a patient with ATL in whom Takotsubo cardiomyopathy and HAM developed after mogamulizumab administration.

Lower sulfurtransferase detoxification rates of cyanide in konzo-A tropical spastic paralysis linked to cassava cyanogenic poisoning.

Using a matched case-control design, we sought to determine whether the odds of konzo, a distinct spastic paraparesis associated with food (cassava) cyanogenic exposure in the tropics, were associated with lower cyanide detoxification rates (CDR) and malnutrition. Children with konzo (N=122, 5-17 years of age) were age- and sex-matched with presumably healthy controls (N=87) and assessed for motor and cognition performances, cyanogenic exposure, nutritional status, and cyanide detoxification rates (CDR). Cyanogenic exposure was ascertained by thiocyanate (SCN) concentrations in plasma (P-SCN) and urine (U-SCN). Children with a height-for-age z-score (HAZNCHS)<-2 were classified as nutritionally stunted. CDR was measured as time required to convert cyanide to SCN, and expressed as ms/µmol SCN/mg protein or as mmolSCN/ml plasma/min. Mean (SD) U-SCN in children with konzo was 521.9 (353.6) µmol/l and was, significantly higher than 384.6 (223.7) µmol/l in those without konzo. Conditional regression analysis of data for age- and sex- matched case-control pairs showed that konzo was associated with stunting (OR: 5.8; 95% CI: 2.7-12.8; p<0.01; N=83 paired groups) and higher U-SCN (OR: 1.1; 95% CI: 1.02-1.20 per 50-µmol increase in U-SCN; p=0.02; N=47 paired groups). After adjusting for stunting and U-SCN, the odds of developing konzo was reduced by 63% (95% CI: 11-85%, p=0.03; N=41 paired groups) for each 5mmol SCN/(ml plasma/min)-increase in CDR. Linear regression analysis indicated a significant association between BOT-2 or KABC-II scores and both the HAZNCHS z-score and the U-SCN concentration, but not the CDR. Our findings provide evidence in support of interventions to remove cyanogenic compounds from cassava prior to human consumption or, peharps, enhance the detoxification of cyanide in those relying on the cassava as the main source of food.

Not the Usual Viral Suspects: Parvovirus B19, West Nile Virus, and Human T-Cell Lymphotrophic Virus Infections After Kidney Transplantation.

Kidney transplant recipients are at increased risk of developing clinical disease due to uncommon opportunistic viral pathogens. Refractory anemia is classically associated with parvovirus B19 infection. West Nile virus has the propensity to cause fever and neurologic symptoms, while spastic paresis and lymphoma can be triggered by human T cell lymphotrophic virus. In this review article, the epidemiology, clinical manifestations, diagnosis and treatment of less common viruses are discussed in the setting of kidney transplantation.

Ankle clonus and thiocyanate, linamarin, and inorganic sulphate excretion in school children in communities with Konzo, Mozambique.

We examined 397 school children for ankle clonus in five communities in three districts affected by konzo, spastic paraparesis associated with cassava consumption. From a subsample of 131 children, we analysed urine specimens for urinary thiocyanate, linamarin, and inorganic sulphate. The proportion of children with clonus varied between sites, ranging from 4 to 22 per cent. Geometric mean thiocyanate, linamarin, and inorganic sulphate concentrations were 163 and 60 mumol/l and 4.4 mmol/l, respectively. Children with ankle clonus had higher urinary thiocyanate concentrations. We recommend prevention to reduce cyanide exposure and further monitoring of cyanide exposure and neurological damage in these communities.

[Successful treatment of acute ethylene glycol poisoning with plasmapheresis]. / Plazmaferézissel sikeresen kezelt akut etilén-glikol mérgezés esete.

The authors show the differential diagnostics, therapy and patomechanism of the ethylene glycol intoxication in connection with review of their young patient became acute paraparetical in consequence of the intoxication. In the therapy of the ethylene glycol intoxication, instead of the traditionally proved hemodialysis their patients was treated with great efficiency with plasmapheresis. The authors show the patomechanism of a patient came with atypical Guillain-Barré-Syndrome. In the hinterground of an acute paraparesis was an ethylene glycol intoxication identified. In the therapy of this intoxication their patients was treated with great efficiency with plasmapheresis instead of the traditionally proved haemodialysis. They touch upon the possibility of monitoring with gas chromatography of the patients plasmapheretised, and call attention the easy check of the sodium fluorescein content of the antifreeze additive therefore the rapid recognition of the intoxicated status.

Thoracic spinal Pantopaque cyst and associated syrinx resulting in progressive spastic paraparesis: case report.

OBJECTIVE AND IMPORTANCE: We describe a unique presentation of a thoracic spinal Pantopaque cyst. Although Pantopaque is no longer used, sequelae of its long-term use may continue to surface. CLINICAL PRESENTATION: Our patient presented to the emergency room with 4 months of progressive lower extremity numbness, spasticity, and incontinence and a 2-day history of left upper extremity dysesthesias. Magnetic resonance imaging at admission revealed a C7-T10 syrinx and an intrathecal extramedullary mass to the right of the spinal cord at T10-T11. INTERVENTION: Radiographic evidence led to the initial diagnosis of hemangioblastoma. The results of a critical analysis of the preoperative studies caused us to suspect a Pantopaque cyst. This suspicion was confirmed at the time of surgery, when a cyst filled with contrast medium was revealed. T9-T11 laminectomies were performed, allowing for a 5-cm dural opening. The cyst was aspirated and excised, and then the syrinx was decompressed. CONCLUSION: Our patient experienced significant sensory and motor improvement postoperatively. We present a previously undescribed complication of one of the most commonly used contrast media, Pantopaque. Despite its replacement with newer agents, it may continue to play a significant role in the pathological presentation of patients in whom it was previously used.

[Neuron damage in the rat spinal cord induced by acromelic acid].

A single systemic injection of acromelic acid, a novel kainate analogue, caused long-lasting spastic paraparesis in the rat. Two rats that developed paraparesis were neuropathologically examined one week and three months after the injection, respectively. Numerous degenerated neurons with marked reactive gliosis were scattered in the gray matter of the spinal cord of the rat with paraparesis for one week. Degenerated neurons were most abundant in the core part of lumbar and sacral segments. The cytometry on the 1st sacral segment disclosed that the number of small neurons was significantly decreased. No morphological sign of neuron damage was demonstrated in the rest of the central nervous system. These pathological changes were responsible for the development of characteristic behavioral changes which were quite different from those induced by kainic acid. The regional difference between the neuron damage induced by acromelic acid and that induced by kainic acid suggests the presence of plural kinds of kainate receptor subtypes in the rat central nervous system. This assumption is supported by receptor binding studies on glutamate receptor subtypes, indicating the low affinity of acromelic acid for both kainate and AMPA binding sites. Acromelic acid may exert its potent depolarizing and neurotoxic effects through activating a new class of kainate receptor subtypes.