Panic Attack, Severe Hypophosphatemia and Rhabdomyolysis in the Setting of a Motor Functional Neurological Disorder.

(1) Background: panic attack is often regarded as a benign disorder with variable physical and psychological symptoms. (2) Case Presentation: We here report the case of a 22-year-old patient known for an episode of motor functional neurological disorder a year earlier who presented a panic attack with hyperventilation causing severe hypophosphatemia and rhabdomyolysis, as well as mild tetraparesis. Electrolyte disturbances quickly resolved after phosphate substitution and rehydration. However, clinical signs suggesting a relapse of a motor functional neurological disorder appeared (improved walking with dual tasks). Diagnostic workup, including brain and spinal magnetic resonance imaging, as well as electroneuromyography and genetic testing for hypokalemic periodic paralysis, was unremarkable. Tetraparesis, lack of endurance, and fatigue eventually improved after several months. (3) Conclusions: the present case report highlights the intertwined relationship between a psychiatric disorder, leading to hyperventilation and acute metabolic disturbances, and functional neurological manifestations.

Precision rehabilitation for aphasia by patient age, sex, aphasia severity, and time since stroke? A prespecified, systematic review-based, individual participant data, network, subgroup meta-analysis.

BACKGROUND: Stroke rehabilitation interventions are routinely personalized to address individuals' needs, goals, and challenges based on evidence from aggregated randomized controlled trials (RCT) data and meta-syntheses. Individual participant data (IPD) meta-analyses may better inform the development of precision rehabilitation approaches, quantifying treatment responses while adjusting for confounders and reducing ecological bias. AIM: We explored associations between speech and language therapy (SLT) interventions frequency (days/week), intensity (h/week), and dosage (total SLT-hours) and language outcomes for different age, sex, aphasia severity, and chronicity subgroups by undertaking prespecified subgroup network meta-analyses of the RELEASE database. METHODS: MEDLINE, EMBASE, and trial registrations were systematically searched (inception-Sept2015) for RCTs, including ⩾ 10 IPD on stroke-related aphasia. We extracted demographic, stroke, aphasia, SLT, and risk of bias data. Overall-language ability, auditory comprehension, and functional communication outcomes were standardized. A one-stage, random effects, network meta-analysis approach filtered IPD into a single optimal model, examining SLT regimen and language recovery from baseline to first post-intervention follow-up, adjusting for covariates identified a-priori. Data were dichotomized by age (⩽/> 65 years), aphasia severity (mild-moderate/ moderate-severe based on language outcomes' median value), chronicity (⩽/> 3 months), and sex subgroups. We reported estimates of means and 95% confidence intervals. Where relative variance was high (> 50%), results were reported for completeness. RESULTS: 959 IPD (25 RCTs) were analyzed. For working-age participants, greatest language gains from baseline occurred alongside moderate to high-intensity SLT (functional communication 3-to-4 h/week; overall-language and comprehension > 9 h/week); older participants' greatest gains occurred alongside low-intensity SLT (⩽ 2 h/week) except for auditory comprehension (> 9 h/week). For both age-groups, SLT-frequency and dosage associated with best language gains were similar. Participants ⩽ 3 months post-onset demonstrated greatest overall-language gains for SLT at low intensity/moderate dosage (⩽ 2 SLT-h/week; 20-to-50 h); for those > 3 months, post-stroke greatest gains were associated with moderate-intensity/high-dosage SLT (3-4 SLT-h/week; ⩾ 50 hours). For moderate-severe participants, 4 SLT-days/week conferred the greatest language gains across outcomes, with auditory comprehension gains only observed for ⩾ 4 SLT-days/week; mild-moderate participants' greatest functional communication gains were associated with similar frequency (⩾ 4 SLT-days/week) and greatest overall-language gains with higher frequency SLT (⩾ 6 days/weekly). Males' greatest gains were associated with SLT of moderate (functional communication; 3-to-4 h/weekly) or high intensity (overall-language and auditory comprehension; (> 9 h/weekly) compared to females for whom the greatest gains were associated with lower-intensity SLT (< 2 SLT-h/weekly). Consistencies across subgroups were also evident; greatest overall-language gains were associated with 20-to-50 SLT-h in total; auditory comprehension gains were generally observed when SLT > 9 h over ⩾ 4 days/week. CONCLUSIONS: We observed a treatment response in most subgroups' overall-language, auditory comprehension, and functional communication language gains. For some, the maximum treatment response varied in association with different SLT-frequency, intensity, and dosage. Where differences were observed, working-aged, chronic, mild-moderate, and male subgroups experienced their greatest language gains alongside high-frequency/intensity SLT. In contrast, older, moderate-severely impaired, and female subgroups within 3 months of aphasia onset made their greatest gains for lower-intensity SLT. The acceptability, clinical, and cost effectiveness of precision aphasia rehabilitation approaches based on age, sex, aphasia severity, and chronicity should be evaluated in future clinical RCTs.

Syndrome of the trephined: clinical spectrum, risk factors, and impact of cranioplasty on neurologic recovery in a prospective cohort.

Syndrome of the trephined (SoT) is an underrecognized complication after decompressive craniectomy. We aimed to investigate SoT incidence, clinical spectrum, risk factors, and the impact of the cranioplasty on neurologic recovery. Patients undergoing a large craniectomy (> 80 cm2) and cranioplasty were prospectively evaluated using modified Rankin score (mRS), cognitive (attention/processing speed, executive function, language, visuospatial), motor (Motricity Index, Jamar dynamometer, postural score, gait assessment), and radiologic evaluation within four days before and after a cranioplasty. The primary outcome was SoT, diagnosed when a neurologic improvement was observed after the cranioplasty. The secondary outcome was a good neurologic outcome (mRS 0-3) 4 days and 90 days after the cranioplasty. Logistic regression models were used to evaluate the risk factors for SoT and the impact of cranioplasty timing on neurologic recovery. We enrolled 40 patients with a large craniectomy; 26 (65%) developed SoT and improved after the cranioplasty. Brain trauma, hemorrhagic lesions, and shifting of brain structures were associated with SoT. After cranioplasty, a shift towards a good outcome was observed within 4 days (p = 0.025) and persisted at 90 days (p = 0.005). Increasing delay to cranioplasty was associated with decreased odds of improvement when adjusting for age and baseline disability (odds ratio 0.96; 95% CI, 0.93-0.99, p = 0.012). In conclusion, SoT is frequent after craniectomy and interferes with neurologic recovery. High suspicion of SoT should be exercised in patients who fail to progress or have a previous trauma, hemorrhage, or shifting of brain structures. Performing the cranioplasty earlier was associated with improved and quantifiable neurologic recovery. Graphical abstract.

[Emotional and behavior disorders after brain lesion]. / Troubles émotionnels et comportementaux après lésion cérébrale.

After a brain lesion, emotional and behavioral disorders affect the quality of life of the patients and their relatives. This article aims to give some cues to manage three problems chosen for their high frequency: apathy, aggression and depression. It will be reviewed how to recognize, to evaluate and to treat them.

Les lésions cérébrales sont fréquemment suivies de troubles émotionnels et comportementaux qui altèrent la qualité de vie du patient et de ses proches. Trois de ces troubles, particulièrement fréquents, sont discutés dans cet article : l'apathie, l'agressivité et la dépression. Une attention particulière sera prêtée à ces symptômes, à leur gravité, ainsi qu'à leur prise en charge, leur traitement médicamenteux ou psychothérapeutique.

RELEASE: a protocol for a systematic review based, individual participant data, meta- and network meta-analysis, of complex speech-language therapy interventions for stroke-related aphasia.

Background: Speech and language therapy (SLT) benefits people with aphasia following stroke. Group level summary statistics from randomised controlled trials hinder exploration of highly complex SLT interventions and a clinically relevant heterogeneous population. Creating a database of individual participant data (IPD) for people with aphasia aims to allow exploration of individual and therapy-related predictors of recovery and prognosis. Aim: To explore the contribution that individual participant characteristics (including stroke and aphasia profiles) and SLT intervention components make to language recovery following stroke. Methods and procedures: We will identify eligible IPD datasets (including randomised controlled trials, non-randomised comparison studies, observational studies and registries) and invite their contribution to the database. Where possible, we will use meta- and network meta-analysis to explore language performance after stroke and predictors of recovery as it relates to participants who had no SLT, historical SLT or SLT in the primary research study. We will also examine the components of effective SLT interventions. Outcomes and results: Outcomes include changes in measures of functional communication, overall severity of language impairment, auditory comprehension, spoken language (including naming), reading and writing from baseline. Data captured on assessment tools will be collated and transformed to a standardised measure for each of the outcome domains. Conclusion: Our planned systematic-review-based IPD meta- and network meta-analysis is a large scale, international, multidisciplinary and methodologically complex endeavour. It will enable hypotheses to be generated and tested to optimise and inform development of interventions for people with aphasia after stroke. Systematic review registration: The protocol has been registered at the International Prospective Register of Systematic Reviews (PROSPERO; registration number: CRD42018110947).

[Neurological consequences and complications of alcohol consumption]. / Conséquences et complications neurologiques de la consommation d'alcool.

In Switzerland, alcohol consumption is often trivialized and widely accepted as normal behavior. However, even in small quantities, repeated alcohol intake can cause injury to both the central nervous system and the peripheral one. Various mechanisms are involved : direct neurotoxicity, metabolic disorders, vitamin deficiencies, systemic injuries (hepatic, cardiovascular, immune), and accidents. This article describes potential neurological complications and their mechanisms, emphasizing the importance of early screenings for abusive consumption.

En Suisse, la consommation d'alcool est une pratique bien ancrée dans les mœurs, considérée comme normale. Cependant, même pris en petites quantités, l'alcool peut provoquer une atteinte du système nerveux, tant central que périphérique. Les mécanismes impliqués sont divers : neurotoxicité directe, désordres métaboliques, carences vitaminiques, atteintes systémiques (en particulier du système hépatique, cardiovasculaire et immunitaire) ou accidents en tous genres. Cet article décrit les complications neurologiques possibles et évoque leurs mécanismes dans l'intention de souligner l'importance d'un dépistage précoce d'une consommation à risque.

The Role of the Cognitive Control System in Recovery from Bilingual Aphasia: A Multiple Single-Case fMRI Study.

Aphasia in bilingual patients is a therapeutic challenge since both languages can be impacted by the same lesion. Language control has been suggested to play an important role in the recovery of first (L1) and second (L2) language in bilingual aphasia following stroke. To test this hypothesis, we collected behavioral measures of language production (general aphasia evaluation and picture naming) in each language and language control (linguistic and nonlinguistic switching tasks), as well as fMRI during a naming task at one and four months following stroke in five bilingual patients suffering from poststroke aphasia. We further applied dynamic causal modelling (DCM) analyses to the connections between language and control brain areas. Three patients showed parallel recovery in language production, one patient improved in L1, and one improved in L2 only. Language-control functions improved in two patients. Consistent with the dynamic view of language recovery, DCM analyses showed a higher connectedness between language and control areas in the language with the better recovery. Moreover, similar degrees of connectedness between language and control areas were found in the patients who recovered in both languages. Our data suggest that engagement of the interconnected language-control network is crucial in the recovery of languages.

[Detection and management of the neurologic dysphagia]. / Détection et prise en charge d'un trouble de déglutition neurologique.

Neurologic dysphagia is frequent and has consequences which can be severe, such as, denutrition andpneumonia. In most cases, it can be detected with a clinical exam. The management includes some general measures, an adaptation of textures, specific rehabilitation, and nutritional assessment to judge whether complementary enteral nutrition is needed.

Radiological signs of the syndrome of the trephined.

INTRODUCTION: Syndrome of the trephined (ST) is a post-craniectomy complication. It is characterized by the appearance of new neurological symptoms following the craniectomy, which are relieved after cranioplasty. The purpose of our work was to identify radiological signs and imaging biomarkers of the ST. METHODS: CT images of 32 patients were retrospectively analyzed (ST = 13, controls = 19). While the shapes of craniectomy flap were qualitatively assessed, deviation of the midline structures, relative intracranial cerebrospinal fluid (CSF) volume, and the 3rd ventricle's volume were quantitatively measured. RESULTS: We did not find between-group differences in the mean age or number of post-craniectomy days. ST was diagnosed during the second post-craniectomy month. The occurrence of a sunken skin flap sign was similar in both groups (69.23 % in ST group, 57.89 % in control group). Occurrence of paradoxical herniation and deviation of the midline structures were not significantly different between groups. Mean relative intracranial CSF volume was significantly smaller in ST patients (ST = 5.59 %, controls = 8.12 %, p = 0.01). ST patients, compared to controls, had also significantly smaller mean 3rd ventricle volumes (ST = 1748 mm(3), controls = 2772.97 mm(3), p = 0.03). CONCLUSIONS: ST is an infrequent and delayed post-craniectomy complication. The most common radiological findings (paradoxical herniation, deviation of the midline structures, and sunken skin flap sign) might not be specific for ST. Significantly lower 3rd ventricle, and relative intracranial CSF volumes, suggest that altered biophysical CSF properties underlie ST pathophysiology. Therefore, volume measurements of 3rd ventricle could be useful for identification of patients who have higher probability of developing the ST.

Recurrent multiple CNS hemangioblastomas with VHL disease treated with pazopanib: a case report and literature review.

Hemangioblastoma is a rare benign neoplasm, accounting for less than 2% of all primitive brain tumors. It may arise sporadically in a solitary form, or associated with Von Hippel-Lindau (VHL) disease with multiple tumors. Surgery is the mainstay treatment, but management is challenging in case of recurrent and/or multiple tumors. VHL protein is defective in both forms of hemangioblastoma, leading to the accumulation of hypoxia-inducible factor, stimulating angiogenesis via VEGF and PDGF mainly. Here, we report a 37-year-old woman's case with recurrent and rapidly progressive VHL-associated hemangioblastomas, causing severe disability. She was treated 24 months with pazopanib, a multityrosine kinase inhibitor (TKI) targeting VEGF and PDGF-ß pathways. Despite moderate radiological changes, progressive improvement in her clinical condition persisting over 3 years was observed. Inhibiting angiogenesis is a therapeutic option that may improve the quality of life and the autonomy of VHL patients disabled with multiple hemangioblastomas.

Facial blindsight.

Blindsight denotes unconscious residual visual capacities in the context of an inability to consciously recollect or identify visual information. It has been described for color and shape discrimination, movement or facial emotion recognition. The present study investigates a patient suffering from cortical blindness whilst maintaining select residual abilities in face detection. Our patient presented the capacity to distinguish between jumbled/normal faces, known/unknown faces or famous people's categories although he failed to explicitly recognize or describe them. Conversely, performance was at chance level when asked to categorize non-facial stimuli. Our results provide clinical evidence for the notion that some aspects of facial processing can occur without perceptual awareness, possibly using direct tracts from the thalamus to associative visual cortex, bypassing the primary visual cortex.

Impairment of semantic memory after basal forebrain and fornix lesion.

Semantic memory impairment is classically associated with lesion of the anterior temporal lobe. We report the case of a patient with severe semantic knowledge impairment and anterograde amnesia after bilateral ischemic lesion of the fornix and of the basal forebrain following surgical clipping of an aneurysm of the anterior communicating artery. Fluorodeoxyglucose positron emission tomography (FDG-PET) showed a temporal hypometabolism. Severe semantic impairment is a rare complication after rupture of an anterior communicating artery aneurysm and may result from disconnection of the temporal lobe.

Anaplastic medullary ependymoma presenting as subarachnoid hemorrhage.

A-41-year old man presented with violent thunderclap headache and a bilateral proprioceptive sensibility deficit of the upper limbs. Cerebral CT scan and MRI were negative. Lumbar puncture confirmed subarachnoid hemorrhage (SAH), but cerebral angiography was negative. Three months later, the patient presented with paraparesis, and a thorough work-up revealed a diffuse, anaplastic extramedullary C7-D10 ependymoma with meningeal carcinomatosis considered the source of hemorrhage. The patient went through a D5-D8 laminectomy, temozolomide chemotherapy, and radiotherapy. The situation remained stable for a few months. In this paper, we would like to emphasize that spinal masses should be considered in cases of SAH with negative diagnostic findings for aneurysms or arteriovenous malformation.

Isolated prospective confabulation in Wernicke-Korsakoff syndrome: a case for reality filtering.

A 57-year-old man suffered severe amnesia and disorientation, accompanied by content-specific confabulation, due to an alcoholic Wernicke-Korsakoff syndrome. For months, he was deeply concerned about a single obligation that he thought he had to respond to, but which he had already assumed 20 years previously. This monothematic, prospective confabulation was associated with failures of reality filtering as previously documented in behaviorally spontaneous confabulation and disorientation: the patient failed to suppress the interference of currently irrelevant memories and to abandon anticipations that were no longer valid (impaired extinction capacity). Magnetic resonance imaging showed damage to the mamillary bodies and the dorsomedial thalamic nucleus. Positron emission tomography (FDG-PET) showed extended orbitofrontal hypometabolism. We suggest that isolated prospective confabulation shares the core feature (acts and thoughts based on currently irrelevant memory), mechanism (failure of reality filtering), and anatomical basis (orbitofrontal dysfunction) with behaviorally spontaneous confabulations.

Changes in oro-facial function and hand-grip strength during a 2-year observation period after stroke.

OBJECTIVES: Recovery from stroke-related oro-facial impairment has rarely been investigated. In this longitudinal study chewing efficiency, maximum lip and bite force as well as masseter muscle thickness were evaluated and compared with hand-grip force. MATERIALS AND METHODS: Thirty-one hospitalized stroke patients and 24 controls were recruited for this cohort study with 2-year follow-up. Chewing efficiency was evaluated with a color mixing ability test, lip forces with a traction dynamometer, bite force with a digital gauge, masseter muscle thickness using ultrasound measurements and grip strength with a hand dynamometer. RESULTS: During the 2-year observation period, patients were evaluated four times. A total of 21 patients dropped out of the study. Stroke patients showed significantly impaired chewing efficiency and lower lip forces than controls with no significant improvement over time. Bite forces were not different between ipsi- and contralesional sides, in contrast to contralesional hand-grip strength which was significantly impaired and did not improve during the observation period. On the first examination with a median of 40 days after stroke, masseter thickness was reduced contralesional, but did not continue to show significant side-differences during follow-up. CONCLUSIONS: Stroke affects the upper limb and the masseter muscles differently on a functional and morphological level. Further research is needed to evaluate the predictive value of oro-facial parameters on functional outcome after stroke. CLINICAL RELEVANCE: Impaired chewing efficiency and reduced lip force are quantifiable symptoms in stroke patients which seem not to improve in absence of oro-facial rehabilitation procedures.

Modulation of environmental reduplicative paramnesia by perceptual experience.

Environmental reduplicative paramnesia (ERP) is characterized by the involuntary attribution of a false identity to a place. ERP has rarely been examined experimentally; its mechanisms therefore remain speculative. Here, we describe a patient with extended traumatic right fronto-temporal damage and severe persistent ERP, in whom we were able to modulate ERP by exposing him to various typical landmarks of the town where he was hospitalized. When landmarks were ambiguous as regards location (e.g., unknown buildings), the patient erroneously localized himself in his hometown, which was more than two thousand kilometers away. In contrast, when he visited distinct landmarks of the place where he actually resided, his ERP was immediately corrected, and spatial orientation was restored. These findings indicate that ERP may be temporarily modifiable through perception of unequivocal topographic information.

Iron-deficiency anemia as a rare cause of cerebral venous thrombosis and pulmonary embolism.

Cerebral venous thrombosis (CVT) is a relatively rare cause of stroke and has a wide spectrum of unspecific symptoms, which may delay diagnosis. There are many etiologies, including hematological disorders, trauma, infection, and dehydration. Iron-deficiency anemia (IDA) has been reported as an extremely rare cause of CVT, especially in adults.