Tolosa-Hunt syndrome relapse during pregnancy following chronic intake of combined oral contraceptives.

Tolosa-Hunt syndrome (THS) is a rare syndrome of painful ophthalmoplegia secondary to an idiopathic granulomatous inflammation affecting the cavernous sinus, superior orbital fissure or orbit. Pregnancy and pregnancy-related hormones have been identified as potential triggers. A 39-year-old gravida-2 para-1 woman with prior chronic intake of combined oral contraceptives (COC) suffered two episodes of painful ophthalmoplegia-the first event with spontaneous remission and the relapse occurring during pregnancy and with complete resolution following steroid treatment. MRI revealed a postinflammatory mass at the junction of the left orbital apex and anterior cavernous sinus, supporting the diagnosis of THS. To our knowledge, this is the first report of a THS relapse occurring during pregnancy following a chronic history of COC intake. This case adds to the growing evidence supporting the relationship between immune and hormonal factors that may be present during pregnancy and the disease pathogenesis of THS.

FIP1L1-PDGFRA-Positive Hypereosinophilia Presenting with Bilateral Extracranial Carotid Artery Aneurysms.

OBJECTIVE: To describe a case of an adult female Filipino with hypereosinophilia and bilateral carotid artery aneurysms who subsequently developed bilateral cerebral hemisphere strokes following aneurysm stenting. CASE DESCRIPTION: A 57-year-old female patient with persistent hypereosinophilia presented with progressively enlarging bilateral neck masses, revealed to be carotid artery aneurysms on computed tomography angiography. Following surgical exploration, she later developed right-sided hemiplegia, aphasia, and right hemianopia. Cranial computed tomography revealed infarcts on both middle cerebral artery territories. Bone marrow biopsy and fluorescent in situ hybridization revealed findings suggestive of hypereosinophilic syndrome. She was started on standard aspirin and statin therapy and was discharged sixteen days after the procedure. Partial improvement of neurologic deficits was noted two months later on follow up. Chemotherapy with imatinib was initiated. CONCLUSIONS: This patient's prothrombotic state from FIP1L1-PDGFRA-positive hypereosinophilia may have led to large carotid artery aneurysm formation and intramural thrombosis. This case demonstrates a possible and heretofore undocumented neurovascular sequela of hypereosinophilic syndrome.

A novel language-neutral Visual Cognitive Assessment Test (VCAT): validation in four Southeast Asian countries.

BACKGROUND: Cognitive screeners are imperative for early diagnosis of dementia. The Visual Cognitive Assessment Test (VCAT) is a language-neutral, visual-based test which has proven useful for a multilingual population in a single-center study. However, its performance utility is unknown in a wider and more diverse Southeast Asian cohort. METHODS: We recruited 164 healthy controls (HC) and 120 cognitively impaired (CI) subjects- 47 mild cognitive impairment (MCI) and 73 mild Alzheimer's disease (AD) dementia participants, from four countries between January 2015 and August 2016 to determine the usefulness of a single version of the VCAT, without translation or adaptation, in a multinational, multilingual population. The VCAT was administered along with established cognitive evaluation. RESULTS: The VCAT, without local translation or adaptation, was effective in discriminating between HC and CI subjects (MCI and mild AD dementia). Mean (SD) VCAT scores for HC and CI subjects were 22.48 (3.50) and 14.17 (5.05) respectively. Areas under the curve for Montreal Cognitive Assessment (0.916, 95% CI 0.884-0.948) and the VCAT (0.905, 95% CI 0.870-0.940) in discriminating between HCs and CIs were comparable. The multiple languages used to administer VCAT in four countries did not significantly influence test scores. CONCLUSIONS: The VCAT without the need for language translation or cultural adaptation showed satisfactory discriminative ability and was effective in a multinational, multilingual Southeast Asian population.

Cognitive Impairment after Mild Stroke: Development and Validation of the SIGNAL2 Risk Score.

BACKGROUND: Post stroke cognitive impairment (PSCI), an important complication of strokes, has numerous risk factors. A scale adequately classifying risk of cognitive impairment 3-6 months after mild stroke will be useful for clinicians. OBJECTIVE: To develop a risk score based on clinical and neuroimaging variables that will be useful in identifying mild ischemic stroke patients at high risk for PSCI. METHODS: The risk score development cohort comprised of a retrospective dataset of 209 mild stroke patients with MRI confirmed infarcts, without pre-stroke cognitive impairment, and evaluated within 6 months post-stroke for PSCI. Logistic regression identified factors predictive of PSCI and a risk score was developed based on regression coefficients. The risk score was checked for stability using 10-fold cross-validation and validated in an independent prospective cohort of 185 ischemic mild stroke patients. RESULTS: Within 6 months post-stroke, 37.32% developed PSCI in the retrospective dataset. A 15-point risk score based on age, education, acute cortical infarcts, white matter hyperintensity, chronic lacunes, global cortical atrophy, and intracranial large vessel stenosis was highly predictive of PSCI with an AUC of 0.829. 10.11% with low scores, 52.69% with moderate scores, and 74.07% with high scores developed PSCI. In the prospective validation cohort, the model had an AUC of 0.776, and exhibited similar accuracy and stability statistics at both 6 and 12 months. CONCLUSION: The seven item risk score adequately identified mild stroke patients who are at an increased risk of developing PSCI.

Montreal Cognitive Assessment for the screening and prediction of cognitive decline in early Parkinson's disease.

BACKGROUND: Early diagnosis of cognitive impairment in PD would allow appropriate monitoring and timely intervention to reduce the progression to dementia (PDD). OBJECTIVE: To study the usefulness of the Montreal Cognitive Assessment (MoCA) in the screening for mild cognitive impairment (PD-MCI) and its predictive utility in determining longitudinal cognitive decline in PD. METHODS: Prospective longitudinal study of patients with mild PD. PD-MCI and PDD was diagnosed based on the Movement Disorder taskforce (MDS) criteria. Receiver Operating Characteristic analyses and Cox regression analyses were performed. RESULTS: 95 patients; mean age 66.37 (SD 7.86); mean H&Y score of 1.99 (SD 0.45) were studied. At baseline, 34 patients fulfilled the MDS criteria for PD-MCI. MoCA, compared to the MMSE had a high discriminatory power in detecting PD-MCI [Area Under Curve (AUC) of 0.912, p < 0.001]. A MoCA score of ≤26 provided a sensitivity of 93.1% for the diagnosis of PD-MCI. In the longitudinal cohort over 2 years, baseline MOCA was useful in predicting cognitive decline (AUC of 0.707, p = 0.05). With Cox regression analyses, a 1-point lower score on baseline MoCA was associated with a 34% increased risk of cognitive decline [Hazard ratio (HR) 1.34; 95% CI: 1.03-1.74: p = 0.029]. A baseline MoCA ≤26 was highly predictive of progressive cognitive decline (HR 3.47, 95% CI: 2.38-5.07; p < 0.01). CONCLUSIONS: MoCA is a reliable tool in predicting cognitive decline in early PD. A MoCA score of ≤26 significantly increases the risk for progressive cognitive decline.

Nonmotor features in sex-linked dystonia parkinsonism.

The natural history of sex-linked dystonia parkinsonism (XDP) has been well documented. However, its nonmotor features have not yet been fully described. We reviewed the available literature on the nonmotor features in XDP. We found five articles involving 79 XDP patients, three of which were on cognition and two on mood (anxiety and depression). There were two case reports showing executive dysfunction. The other paper showed impairments in abstract thinking and motor programming. Two articles were on mood (anxiety and depression). The prevalence of anxiety symptoms was 16.7% and 54.8-92.9% had depressive symptoms. The identification of these nonmotor features should lead to early and appropriate management.

Suicide among patients with X-linked dystonia parkinsonism (XDP): A retrospective study

OBJECTIVE: To describe the rate of suicide and explore its possible related factors among patients with X-linked Dystonia Parkinsonism. Specifically, this paper aimed to describe the rate of suicide among patients with XDP based on the Philippine XDP registry and to describe these patients in terms of severity of XDP and psychosocial factors. BACKGROUND: Chronic progressive neurologic conditions have been associated with serious psychosocial stresses. Suicide among patients with X-linked Dystonia Parkinsonism has been previously reported to be high. METHODS: A retrospective chart review was done on XDP patients with deaths attributable to suicide. XDP related variables and available psychosocial factors were noted. RESULTS: The prevalence of suicide among all XDP patients registered is 4.16%. There are 194 deaths in the Philippine XDP registry, 21 of which were attributable to suicide, a proportion of 10.8%. Their mean age at suicide was 44, around 7.76 mean years from the onset of illness. All of the patients were either in generalized dystonia or parkinsonian stage when they had suicide. Psychosocial variables noted were marital and family conflict, and loss of employment. None of the patients had a prior documented psychiatric illness but several of them showed symptoms of depression prior to suicide. CONCLUSIONS: There is a high rate of suicide among patients with XDP which is comparable to other disabling neurodegenerative diseases. It occurs relatively late in the course where the patient is already in the stage of generalized dystonia or parkinsonism. Possible psychosocial risks include poor family support, marital conflict, loss of employment and symptoms suggestive of depression. The present understanding is that depression and suicide in XDP is more likely reactive to the disease rather than part of its clinical feature. This study supports this view.