Case Reports in Neurological Medicine最新文献

Restless arm syndrome (RAS) is an uncommon neurological condition that is characterized by an irresistible urge to move the upper limbs, often worse at rest and transiently relieved by movement. RAS is an upper limb variant of restless legs syndrome (RLS), a more common neurological condition, also known as Willis-Ekbom disease. In view of its atypical presentation, RAS is often underdiagnosed. RAS is reported to share the same risk factors as RLS with its prevalence increasing with age, in the presence of certain conditions, and associated with the use of medications like antipsychotics and antidepressants. A case of possible RAS is described here in a patient who had experienced a few years of RLS-like symptoms and had been on various antidepressants for his depression. His symptoms markedly improved with a trial of gabapentin, which subsequently raised the possibility of RAS as a diagnosis. Clinicians should consider RAS as a differential in patients who experience symptoms suggestive of RLS in the presence of associated risk factors.

A man in his 50s experienced novel, continuous, and progressive headache and neck pain prior to the onset of left-sided peripheral facial nerve palsy. Sequential palsies of left lower Cranial Nerves IX and XII followed. Imaging showed spontaneous cervical artery dissection (sCeAD) of the ipsilateral internal carotid artery. Lower cranial nerve palsies in sCeAD are a frequent result of a local mass effect exerted by the formation of a mural hematoma. The only close topographical relationship between the facial nerve and the internal carotid artery is within the petrous part of the temporal bone but still separated in two different bony canals (facial canal and carotid canal). Thus, a mural hematoma of an internal carotid artery dissection could not cause compression of the facial nerve. In the rare case of facial nerve palsy due to sCeAD, hypoperfusion of the vasa nervorum is the most likely cause. As sCeAD is one of the main reasons for stroke in the youth, it is critical to know and identify potential red flags in patients with peripheral facial nerve palsy, which should lead to additional vascular imaging.

Background: Neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein-associated disease (MOGAD) are central nervous system (CNS) demyelinating disorders characterized by autoantibodies targeting aquaporin-4 (AQP4) and MOG, respectively. Although dual positivity for AQP4-IgG and MOG-IgG antibodies is uncommon, it poses significant diagnostic and therapeutic challenges due to its complex clinical features and uncertain prognosis.

Case presentation: We present the first reported case in the literature of a patient with metastatic renal carcinoma who tested positive for both AQP4 and MOG antibodies. A 49-year-old man with a history of metastatic renal carcinoma experienced progressive neurological symptoms, initially attributed to tumor progression. However, after further investigation, including lumbar puncture and autoantibody testing, a demyelinating process with dual seropositivity for AQP4-IgG and MOG-IgG was identified.

Treatment and outcomes: The patient was treated with high-dose corticosteroids, followed by rituximab, resulting in clinical and radiological stability.

Conclusion: This case highlights the rare occurrence of dual seropositivity for AQP4 and MOG antibodies in a patient with a history of metastatic renal carcinoma, which poses diagnostic and treatment challenges. Although the pathogenesis remains unclear, factors such as genetic predisposition and autoimmune coactivation may contribute to triggering this autoimmune response. This case emphasizes the importance of personalized treatment and the need for further research to optimize management strategies for patients with this complex condition.

Background: Upper airway obstruction secondary to bilateral vocal cord paralysis is not a known classic presentation of bilateral medial medullary infarction (BMMI). This may potentially confound the diagnostic approach, particularly when coexisting with bulbar symptoms and quadriplegia. Prompt recognition is essential for timely and appropriate airway management and subsequent treatment.

Case presentation: A 74-year-old female presented with a three-week stepwise progression of asymmetric quadriparesis, slurred speech, and a prominent biphasic stridor. Flexible fiberoptic laryngoscopy revealed bilateral vocal cord palsy in the median-paramedian position, and an emergency tracheostomy was performed. Magnetic resonance imaging (MRI) of the brain revealed the characteristic "heart shaped" diffusion-weighted imaging (DWI) pattern of BMMI, while magnetic resonance angiography (MRA) exhibited absent flow-related signals in the right vertebral artery. Secondary stroke prevention with clopidogrel was started. However, the patient developed severe pneumonia with massive pleural effusion and expired on the sixth day of hospitalization due to Type 1 respiratory failure.

Conclusion: Bilateral vocal cord paralysis may occur in BMMI, and recognizing this rare association is crucial for timely diagnosis and treatment. The intricate neurovascular anatomy of the medulla may find insight into the rarity of this association.

Cervical spondylotic myelopathy (CSM) is a common cause of spinal cord dysfunction. Because its symptoms may resemble those of intracranial tumors, patients can be misdiagnosed and undergo inappropriate spinal procedures. We describe three patients initially treated with cervical decompression under the impression of CSM. In each case, neurological deficits failed to improve, or even progressed, despite adequate surgery. Further investigation with brain MRI disclosed large meningiomas located in the frontoparietal or parasagittal regions. All tumors were completely resected, pathology confirmed WHO Grade I meningioma, and the patients showed meaningful neurological recovery. These observations remind us that neurological findings must be interpreted in parallel with cervical imaging. A brain MRI should be obtained whenever clinical features are disproportionate to spinal pathology, extend beyond the usual pattern of myelopathy, or remain unresolved after decompression.

West Nile virus (WNV) is the most common mosquito-borne infection in North America; while most cases are asymptomatic, fewer than 1% develop neuroinvasive disease with significant morbidity and mortality. We report a 57-year-old man from rural Wisconsin who presented with a 10-week history of progressive asymmetric quadriparesis and severe intractable pain, preceded by fatigue, shoulder pain, and paresthesias. Neurologic examination demonstrated mild encephalopathy, bulbar involvement, and mixed upper and lower motor neuron signs. MRI showed patchy thoracic cord T2 hyperintensities and diffuse lumbar ventral root enhancement. Electrodiagnostic studies revealed diffuse active denervation and reduced compound muscle action potentials, initially raising concern for amyotrophic lateral sclerosis. Elevated WNV IgM and IgG titers in serum and cerebrospinal fluid confirmed neuroinvasive WNV infection. Despite treatment with corticosteroids and intravenous immunoglobulin, the patient deteriorated and was transitioned to hospice care. Autopsy demonstrated T-cell-mediated meningoencephalitis with widespread lymphocytic inflammation involving motor neurons, spinal cord, ventral rootlets, and peripheral nerves, consistent with diffuse axonopathy. This case underscores that neuroinvasive WNV may closely mimic motor neuron disease and emphasizes the importance of serologic testing for accurate diagnosis. Management remains supportive, and outcomes can be severe due to extensive central and peripheral nervous system involvement.

Beta-adrenergic blockers are effective in migraine prevention but can induce neuropsychiatric side effects, including vivid dreams and nightmares. Their lipophilicity allows penetration of the central nervous system, where β1-adrenergic blockade may disrupt REM sleep, alter noradrenergic activity, and suppress melatonin secretion, contributing to emotionally intense dreams. While reported in cardiovascular patients, this adverse effect remains underrecognized in migraine therapy. Adult patients were identified from those seen in the outpatient department of the Clinical Hospital of University of Chile in Santiago between 2022 and 2024. We present three cases of patients with episodic migraine with aura who developed distressing, recurrent nightmares after initiating propranolol, or metoprolol. Symptoms emerged shortly after treatment initiation and resolved upon discontinuation. Nightmare content involved emotionally distressing themes, leading to significant psychological discomfort. Clinicians should be aware of vivid nightmares as a potential adverse effect of lipophilic beta-blockers in migraine prevention. Understanding this may enable patients to tolerate the symptom. If it impacts adherence and/or quality of life, a treatment change will be needed.

The introduction of computed tomography (CT) myelography for spinal diseases has allowed the diagnosis of several intradural and extradural etiologies. With the advent of water-soluble, nonionic contrast agents, the safety and availability of this technique have expanded. Although magnetic resonance imaging (MRI) has faded the indications for CT myelography, some specific conditions and settings still benefit from the functional flow of contrast in the subarachnoid space and/or the patient's particular limitations in performing MRI (especially in the presence of intense metallic artifacts). We present the case of a 23-year-old male patient who underwent long-term follow-up for an intramedullary epidermoid cyst. At the time of diagnosis, the patient complained of right lower limb tremor and pain, which progressed to leg weakness, with no congenital abnormality. His first surgery resulted in a mass resection without spinal fixation. Three years later, thoracic canal stenosis and kyphosis were diagnosed, leading to a second surgery, consisting of laminectomy and cervicothoracic fixation. At 8 years of age, worsening weakness and sphincter issues prompted further evaluations. CT myelography revealed upper thoracic cord enlargement. An intramedullary epidermoid cyst was diagnosed, and the patient underwent a new gross total resection. CT myelography is not obsolete. Patients requiring spine imaging for oncologic control and/or new or worsening neurological symptoms may benefit from CT myelography when standard spine MRI cannot be performed. Epidermoid cysts require long-term postoperative follow-up, as recurrence may occur years after surgical resection owing to their indolent, benign behavior.

Fahr's syndrome is a rare, slowly progressive neurodegenerative disorder characterised by bilateral cerebral calcifications, mostly in the basal ganglia. These cerebral calcifications are composed of calcium and phosphate and are the result of disturbances in calcium-phosphate homeostasis. The clinical manifestations include neurological, neurocognitive and psychiatric symptoms. This article describes three rare cases of pronounced bilateral cerebral calcifications. All three patients were admitted to the hospital due to a first-time epileptic seizure. In all three cases, laboratory tests showed significant hypocalcaemia, and cerebral computed tomography showed pronounced bilateral cerebral calcifications in various brain areas. After calcium substitution and anticonvulsant treatment, the patients returned to their prehospital condition and were discharged home seizure free. The aim of this article is to highlight the clinical importance of long-term follow-up biochemical laboratory testing and neurocranial imaging in high-risk patients (e.g., after thyroidectomy) to prevent avoidable neurological and psychiatric complications through pharmaceutical and nutritional substitution therapy.

Treatment for chronic myeloid leukemia (CML) with tyrosine kinase inhibitors (TKIs), especially nilotinib and ponatinib, has been associated with atheromatic vascular adverse events including cerebrovascular disease. Herein, we present two patients with CML and long-term nilotinib treatment, who developed severe carotid atherosclerotic stenoses, both extra- and intracranial, resulting in ischemic stroke. The clinical and radiological findings as well as the possible pathophysiological mechanisms of these clinically significant complications are discussed. It seems that new-generation TKIs such as nilotinib, ponatinib, and, to a far lesser extent, bosutinib increase the incidence of vascular occlusive events compared to imatinib, in a dose- and duration-dependent manner. The mechanisms leading to vasculopathy are various and comprise promoting a prothrombotic platelet state, the dysregulation of glucose and lipid metabolism, increase of inflammatory cytokines, and affecting vessel wall endothelial cells. Regarding the outcome of cerebrovascular events, it seems that the discontinuation of TKIs alone or switching to a safer one is insufficient to resolve the stenoses of the cerebral arteries, even under dual antiplatelet treatment, anticoagulation, or high-potency statin therapy. Thus, revascularization strategies such as extracranial to intracranial bypass surgery or stenting should be considered, especially when there is no improvement with medical treatment. These observations expand our knowledge on the association between TKIs and cerebral vascular disease, as well as provide more insights into the underlying pathogenesis. TKIs should not only be selected based on disease-related variables but also based on patient-related factors such as cardiovascular comorbidities.