Case Reports in Neurology最新文献

Background: Tapia's syndrome is a rare neurological condition defined by concurrent unilateral paralysis of the vagus (cranial nerve X) and hypoglossal (cranial nerve XII) nerves. It is most commonly reported as an iatrogenic complication of procedures involving airway manipulation, such as orotracheal intubation. This report describes a unique case of Tapia's syndrome with a temporal association to the initiation of noninvasive continuous positive airway pressure (CPAP) therapy.

Case presentation: A 66-year-old female presented with a four-day history of acute-onset dysphonia, dysphagia, and right-sided tongue deviation. Her symptoms began shortly after initiating CPAP therapy with a full-face mask for newly diagnosed obstructive sleep apnea. She had also recently received multiple vaccinations. Clinical examination revealed right-sided vagus and hypoglossal nerve palsies, and laryngoscopy confirmed right vocal cord paralysis. Extensive diagnostic evaluation, including magnetic resonance imaging and angiography of the brain and neck, effectively excluded central nervous system pathologies such as stroke, demyelinating disease, or mass lesions and diagnosis of Tapia's syndrome was made. The patient was managed by discontinuing CPAP and administering a course of oral corticosteroids, alongside speech and swallowing therapy. She experienced a near-complete resolution of her symptoms over 6 weeks.

Conclusion: This case suggests that Tapia's syndrome can be a rare complication of noninvasive airway support. A multifactorial etiology involving mechanical nerve compression from the CPAP apparatus, potentially compounded by an immune-mediated nerve sensitization from recent vaccinations, should be considered in the differential diagnosis of lower cranial neuropathies.

Introduction: Rapidly progressive para- or tetraparesis and early loss of F-waves on electrodiagnostic studies usually indicate acute inflammatory demyelinating polyneuropathy. A less common alternative etiology is acute spinal cord pathology.

Case presentation: We describe here a case of a 65-year-old woman with progressive motor deficits and absent F-waves early in the disease course due to anterior spinal artery syndrome. We provide serial electrodiagnostic findings with a focus on the basic electrodiagnostic parameters as well as imaging correlates during the inpatient management.

Conclusion: Anterior spinal artery syndrome represents an uncommon cause of absent F-waves on the electrodiagnostic exam. Absent F-waves may precede any changes on the imaging and follow-up investigations are often warranted, as spinal cord ischemia can be a dynamic process.

Introduction: Transient loss of consciousness (TLOC) refers to the sudden, temporary disturbance of consciousness followed by spontaneous recovery. Little is known about acute changes in intracranial pressure (ICP) as a possible cause of TLOC.

Case presentation: We present the case of a 78-year-old man with recurrent episodes of TLOC due to raised ICP triggered by mechanical compression of a large postoperative lumbar pseudomeningocele. The video-electroencephalogram (EEG) showed sudden flattening distinctly different from EEG changes seen in cardiovascular TLOC.

Conclusion: Our EEG observations suggest that a sudden increase in ICP should be considered as a potential cause of TLOC, particularly after spinal surgery.

Introduction: Psychogenic nonepileptic seizures (PNES) are episodes resembling epileptic seizures but not caused by abnormal electrical brain activity. They are part of functional neurological disorders, which disproportionately affect women and are often triggered by a combination of biological, psychological, and social factors. Early recognition is crucial to prevent unnecessary interventions.

Case presentation: We report a 35-year-old woman with a 3-year history of recurrent loss of consciousness, occurring approximately every 15 days, usually coinciding with ovulation and menstruation. Previous cardiology and gynecology evaluations, imaging, blood tests, and EEG were unremarkable. During the most severe episodes, she required intensive care unit admission, intubation, and sedation. A video-EEG with verbal suggestion reproduced a typical event, showing no epileptiform activity. The patient was diagnosed with PNES. Psychotherapy was initiated, and antiepileptic drugs were gradually withdrawn. This, along with patient education, family involvement, and outpatient follow-up, led to a marked reduction in episode frequency.

Conclusion: This case highlights the complex interplay between hormonal fluctuations, reproductive concerns, psychosocial stressors, and gender-specific factors in triggering PNES. Early diagnosis, a multidisciplinary approach, and education of patients and healthcare providers are key to improving outcomes and avoiding unnecessary interventions. To our knowledge, this is the first report describing PNES episodes linked to both menstrual cycle-related stress and reproductive concerns, emphasizing the importance of a gender-sensitive approach.

Introduction: Cryptococcosis is a fungal infection with frequent involvement of the central nervous system, particularly in patients immunocompromised by the human immunodeficiency virus (HIV). Its essential neurological presentation is meningoencephalitis, with exceptional spinal cord involvement. We present the case of an immunocompetent patient with myelitis and vasculitis caused by Cryptococcus.

Clinical case: A 59-year-old male patient with diabetes mellitus presented with 2 weeks of lower limb weakness, vertigo, and urinary retention with subsequent loss of alertness. Magnetic resonance imaging confirmed a longitudinally extensive transverse myelitis, venous sinus thrombosis, and multiple cerebral infarcts involving the cerebellum, basal ganglia, and corpus callosum. Cerebrospinal fluid confirmed Cryptococcus fungal infection. Differential diagnoses (infectious, autoimmune, metabolic, demyelinating, and neoplastic) were excluded. Liposomal amphotericin and flucytosine treatment were started for 6 weeks, with partial improvement. Late primary immunodeficiency was diagnosed based on a low CD4+ count, excluding HIV in multiple instances. The patient continues with motor and sensory sequelae and hypoacusis.

Discussion: Myelitis of infectious origin is predominantly viral and bacterial. Spinal cord involvement by Cryptococcus is extremely unusual, and the literature is limited to case reports. This condition shows high heterogeneity in its presentation, being predominant in patients with immunocompetence. It can be associated with cryptococcomas and transverse or longitudinally extensive myelitis involving any spinal cord segment. The treatment duration and use of corticosteroids are still debatable. Few similar cases have been reported.

Conclusion: Cryptococcus fungal infection should be included in the differential diagnosis of infectious myelopathies, even in patients without HIV infection.

Introduction: Cerebral amyloid angiopathy (CAA) is a vasculopathy characterized by amyloid-beta (Aβ) deposition in the walls of the leptomeningeal and cortical blood vessels. In a minority of patients with CAA, the presence of Aβ deposition triggers an autoimmune inflammatory reaction, referred to as CAA-related inflammation (CAA-ri). It can present in two forms, either as perivascular CAA-ri or as Aβ-related angiitis. The mechanism underlying CAA-ri remains unclear. Symptoms are subacute mental disorders and behavioral or cognitive changes, headaches, seizures, and focal neurological deficits. Most commonly, CAA-ri is a monophasic illness. Relapses occur often years after the initial presentation and are correlated with corticosteroid tapering or immunosuppression cessation. Treatment is often prolonged but effective. We present a rare and instructive case with CAA-ri characterized by rapid cognitive decline, therapy resistance, and fatal outcome and discuss current literature.

Case presentation: We describe a case of an 81-year-old female who presented with progressive confusion, behavior alterations, recurrent falls, and vomiting for 2 weeks. There was a rapid cognitive decline and fatal outcome. The diagnosis of a probable CAA-ri was made based on the clinical presentation and brain MR imaging. To allow long-term corticosteroid replacement, azathioprine was initiated for second-line immunosuppression.

Conclusion: This case report of CAA-ri describes the diagnostic and therapeutic challenges in an elderly patient with rapid cognitive decline. It highlights the severe nature of the condition, the limitations of available treatments, and the importance of early recognition based on the diagnostic criteria and MR imaging and the start of the therapy, while acknowledging that outcomes may remain poor despite intervention.

Introduction: Epilepsy surgery remains underutilized despite strong evidence supporting its efficacy for appropriately selected patients with drug-resistant epilepsy (DRE).

Case presentation: We describe the case of a 23-year-old woman with a 4-year history of refractory focal impaired awareness seizures, experiencing up to four episodes per week despite multiple trialed antiseizure medications at tolerated doses. Initial investigations including magnetic resonance imaging (MRI), EEG, and paraneoplastic screening were unremarkable, and she was managed as having non-lesional left temporal lobe epilepsy. As part of an artificial intelligence driven research initiative, a natural language processing based random forest algorithm reviewing neurology clinic documentation identified her as a potential surgical candidate. Complementary large language model analysis supported extraction of relevant seizure and imaging information. This prompted re-evaluation of the case, with PET demonstrating left temporal hypometabolism and repeat MRI revealing a previously unrecognized encephalocele. She subsequently underwent left temporal lobe polectomy with encephalocele disconnection. The procedure was successful, and she has remained seizure-free for 1 year postoperatively, allowing initiation of ASM weaning and the possibility of long-term cure.

Conclusion: This case illustrates the transformative potential of epilepsy surgery for individuals with DRE and the promising role of artificial intelligence augmented triage systems in reducing delays to surgical referral. Given the morbidity, mortality, and economic burden associated with DRE, timely surgical evaluation should be considered the standard of care for eligible patients. Integrating artificial intelligence tools into clinical workflows may help overcome longstanding barriers to access and ensure that life-changing interventions are offered earlier in the disease course.

Introduction: Mineralizing angiopathy is a rare cause of pediatric stroke and has not previously been described in a Nordic European country. This case highlights the diagnostic value of CT imaging and the potential underdiagnosis of this condition in high-income countries.

Case presentation: An 11-month-old previously healthy boy presented with acute left-sided hemiparesis, with a pediatric National Institutes of Health Stroke (pedNIHSS) score of nine. Initial CT revealed bilateral basal ganglia calcifications and tortuous internal carotid arteries. MRI confirmed an acute infarction in the right basal ganglia. The diagnosis of mineralizing angiopathy was made based on the neuroimaging findings, with sagittal CT views visualizing calcifications following the contours of deep perforating vessels. An extensive stroke workup, including cardiac and metabolic evaluations, was unremarkable. The patient received supportive care, was discharged with a pedNIHSS of five, and is under neurological follow-up.

Conclusion: Mineralizing angiopathy may be underrecognized in pediatric stroke, particularly in settings where MRI is preferred over CT. Early CT imaging can aid diagnosis. Longitudinal follow-up may offer novel insights into the pathophysiology of vascular calcifications.

Introduction: Partially thrombosed aneurysms of the posterior cerebral artery (PCA), especially in the P2 segment, pose considerable treatment challenges owing to complex anatomy and ischemic risk.

Case presentation: We report a 49-year-old woman with a partially thrombosed dissecting P2 aneurysm treated using selective occipital artery-PCA bypass guided by digital subtraction angiography-magnetic resonance fusion imaging, followed by endovascular occlusion using Target Tetra coils. Postoperative imaging confirmed bypass patency and complete aneurysm obliteration without infarction or neurological deficit.

Conclusion: We highlight a feasible treatment strategy for complex PCA aneurysms, emphasizing the importance of fusion imaging and targeted embolization in minimizing complications and preserving function.

Introduction: Primary progressive aphasia (PPA) is a neurodegenerative disorder that causes a gradual decline in language function. While combining transcranial direct current stimulation (tDCS) with rehabilitation of speech and language disorders (SLD rehab) has shown promise, its effect on motor speech disorders such as dysarthria and apraxia of speech (AOS), common in nonfluent variant PPA (nfvPPA), has been unclear.

Case presentation: This study used an N-of-1 crossover design to investigate the effects of SLD rehab-tDCS on articulation and vocalization in a 77-year-old male patient with nfvPPA, dysarthria, and AOS. In the intervention phase, intervention A (anodal tDCS over the left inferior frontal gyrus from the precentral regions, combined with SLD rehab) was compared with intervention B (sham stimulation with SLD rehab) across both short-term (single session) and long-term (12 sessions over 6 weeks) phases, with intervention A preceding B. In both the short- and long-term phases, the assessments of articulation and vocalization showed greater improvement following intervention A. The long-term intervention also led to improvements in general aphasia severity. Furthermore, brain perfusion SPECT imaging revealed increased blood flow in the left fronto-subcortical network.

Conclusions: These preliminary findings from a single case suggest that SLD rehab-tDCS may have the potential to improve not only language but also speech motor functions in nfvPPA.