Case Reports in Neurological Medicine最新文献

Hemichorea is a rare manifestation of ischemic stroke whose lesion is typically located in the contralateral basal ganglia. Its pathomechanism has not been elucidated completely; however, it may be related to nigrostriatal dysfunction. In patients with hemichorea, dopamine transporter-single photon emission computed tomography (DAT-SPECT) reportedly displayed decreased tracer accumulation in the contralateral striatum. Moreover, in exceptional cases, responsible lesions were located in the ipsilateral cerebral hemisphere. This case report describes an 84-year-old man who presented with three weeks of intermittent, involuntary, and twisting movements in his right limbs. On physical examination, the patient had right-sided hemichorea without other neurological deficits. The choreic movements were more frequent in the lower limb than in the upper and provoked when he tried to take a certain posture or engaged in mental arithmetic. Magnetic resonance imaging performed on suspicion of stroke detected a right hemispheric subacute infarct in the posterior part of the lenticular nucleus and posterior limb of the internal capsule. Furthermore, DAT-SPECT revealed decreased tracer accumulation in the right striatum. He was administered oral antiplatelet medication after being diagnosed with lacunar infarction. The choreic movements gradually reduced over the next 8 months and eventually disappeared. The lesion in the lenticular nucleus and internal capsule was considered to have induced ipsilesional hemichorea, considering the temporal proximity between the hemichorea and ischemic stroke. Although DAT-SPECT findings in patients with ipsilesional hemichorea have not been reported, this case suggests that nigrostriatal dopamine dysfunction can contribute to the pathogenesis of ipsilesional hemichorea.

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a neurological emergency typically associated with hypertension or drug toxicity. Although mirtazapine is not a classical serotonergic agent, overdose may induce serotonin syndrome, which can contribute to PRES. Case Presentation: A 50-year-old woman presented with seizures, impaired consciousness, and autonomic instability following ingestion of > 300 mg mirtazapine. Magnetic resonance image (MRI) revealed vasogenic edema in the parieto-occipital and frontal lobes. Her clinical features fulfilled the Hunter criteria for serotonin syndrome. Treatment with cyproheptadine led to full clinical and radiological recovery. Discussion: Serotonin syndrome may disrupt cerebral autoregulation and impair endothelial integrity, contributing to PRES. Although rare, similar cases have been reported with other serotonergic agents. This is the first reported case of mirtazapine overdose resulting in serotonin syndrome-associated PRES. Conclusion: Clinicians should recognize that mirtazapine overdose can cause serotonin syndrome and secondary PRES. Early identification and serotonin antagonism are crucial for recovery and prevention of sequelae.

Pelvic congestion syndrome (PCS) is an underdiagnosed but not rare cause of chronic pelvic pain, affecting approximately 10%-30% of women of reproductive age. It is characterized by venous insufficiency and dilation of the ovarian and pelvic veins, often presenting with symptoms that worsen during menstruation or prolonged standing, and improve in the supine position. Dyspareunia and a sensation of pelvic heaviness are also frequently reported. Neurological manifestations-such as pudendal or femoral nerve irritation-are rare but may offer key diagnostic clues. We present a case of a 30-year-old woman with right-sided pelvic pain radiating to the groin and proximal thigh, consistent with neural irritation. Magnetic neurography revealed dilated pelvic veins in close proximity to the right psoas muscle and the L5 nerve root, suggesting perineural venous engorgement. Selective venography confirmed bilateral ovarian vein insufficiency, and the patient underwent successful embolization with Ruby coils and adjunct sclerotherapy. Postoperative recovery was uneventful, with complete resolution of symptoms. Follow-up at 1 year showed no recurrence, and the patient later achieved a successful pregnancy. This case highlights the potential for pelvic venous congestion to mimic or cause neural symptoms and emphasizes the diagnostic value of magnetic neurography in complex pain presentations. Endovascular treatment proved safe and effective, even in cases with neurological involvement.

Autoimmune psychosis criteria have been proposed for autoimmune encephalitis with prominent psychiatric symptoms as an alternative to biomarker-based diagnostic approaches such as the Graus criteria. We present a case of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis that was initially misdiagnosed as a psychiatric disorder due to serial negative MRI findings and subsequently re-evaluated correctly using autoimmune psychosis criteria. A 15-year-old male developed sudden-onset generalized convulsive seizures that increased progressively in frequency and fluctuating psychiatric symptoms that gradually worsened to include reduced reactivity, language deterioration, and catatonia. On admission, both brain MRI and cerebral spinal fluid (CSF) findings were unremarkable; however, autoimmune encephalitis was strongly suspected based on autoimmune psychosis criteria and subsequently confirmed by detection of oligoclonal bands (OCBs) and anti-NMDAR antibodies in the serum and CSF. Repeated steroid pulse therapy resulted in significant clinical improvement. The patient met multiple autoimmune psychosis criteria, including subacute onset of psychiatric symptoms, catatonia, disproportionate cognitive dysfunction, decreased level of consciousness, and the emergence of seizures. These features are not typically present in primary psychiatric disorders. Anti-NMDAR encephalitis can present with a variety of symptoms, complicating its differentiation from primary psychiatric conditions. The application of autoimmune psychosis criteria may serve as a valuable diagnostic aid, particularly when MRI findings are repeatedly negative.

Subarachnoid hemorrhage (SAH) is a life-threatening condition most commonly caused by aneurysmal rupture. Sentinel headaches, often described as the "worst headache of life" or a "thunderclap headache," are critical warning signs that may precede SAH. However, atypical headaches can complicate early diagnosis. Oculomotor nerve palsy, though rare, may occur as a complication of both aneurysmal and nonaneurysmal SAH. We report a unique case of a 64-year-old woman who initially presented with atypical headache followed by isolated oculomotor nerve palsy, preceding the onset of a nonaneurysmal SAH. This case highlights isolated oculomotor palsy as a potential unrecognized sentinel sign of SAH.

Pseudovestibular syndrome refers to central pathologies that mimic acute unilateral peripheral vestibulopathy, often posing a diagnostic challenge, particularly when key symptoms indicating a central origin are absent. The most common etiology is brain ischemia resulting from posterior inferior cerebellar artery occlusion. This article presents a rare case of a left paramedian cerebellar hemorrhage initially misdiagnosed as right-sided vestibular neuritis. Cerebellar hemorrhage can induce pseudovestibular syndrome by disrupting the connective fibers from the flocculus to the ipsilateral vestibular nucleus in the pons. Additionally, central pathologies affecting the vestibular system may occasionally manifest a pathological vestibulo-ocular reflex. This case report underscores the importance of considering potentially severe central-origin conditions in the differential diagnosis of seemingly benign unilateral peripheral vestibulopathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) following viral infections and influenza vaccination has been well documented. However, there have been no confirmed natural influenza A infections leading to development of CIDP. Therefore, we present the case of a 6-year-old male who developed CIDP following a confirmed influenza A infection. Initially presenting with typical flu-like symptoms, the patient experienced a gradual onset of gait instability and leg weakness approximately 1 month later. Despite initial improvement with intravenous immunoglobulin therapy following a diagnosis of Guillain-Barré syndrome, his symptoms relapsed, including lower extremity weakness, incontinence, and sensory loss. Electromyography confirmed a demyelinating polyneuropathy, leading to a diagnosis of CIDP.

We report the case of a 55-year-old right-handed female with a medical history of hypothyroidism and gastric bypass surgery. The patient initially presented with cognitive impairment, dizziness, and unsteady gait. Despite an unremarkable stroke workup, her symptoms progressed rapidly within 2 days, leading to subsequent admissions and a complex diagnostic journey revealing Susac syndrome-a rare autoimmune disorder affecting the brain's microvasculature, retina, and cochlea. The patient's treatment involved aggressive immunosuppression with corticosteroids, IVIG, mycophenolate, and cyclophosphamide. The patient responded well and had progressive improvement, with discharge to home. This case highlights the diagnostic challenges and management strategies for Susac syndrome.

Dengue fever, caused by a flavivirus transmitted through Aedes mosquitoes, presents a spectrum of clinical manifestations ranging from mild to severe. Several neurological complications, including encephalopathy and encephalitis, have been increasingly recognized. Here, we report a case of central venous thrombosis (CVT) as a postinfectious complication of dengue fever. A 38-year-old previously healthy male presented with classic dengue symptoms and later developed persistent headaches and visual disturbances. Neurological examination revealed papilledema, and imaging confirmed CVT involving the superior straight sinus. Prompt initiation of anticoagulant therapy led to gradual improvement in neurological symptoms and partial recanalization of the thrombosed sinus. Our case underscores the importance of considering thrombotic complications in dengue infections, despite the predominance of hemorrhagic manifestations. Understanding pathophysiology and appropriate management of thrombotic events in dengue fever is crucial for favorable patient outcomes.

Biotinidase deficiency is a rare treatable metabolic disorder caused by biallelic mutations in the BTD gene. In the absence of neonatal screening and treatment, affected children develop typically optic atrophy, hypotonia, early onset seizures, developmental delay, and cutaneous manifestations. Some patients may have atypical presentations mimicking a demyelinating disorder of the central nervous system. We report on the first genetically confirmed Tunisian patient with biotinidase deficiency who presented initially with cutaneous manifestations misdiagnosed as dermatophytosis and subsequently with an opticospinal syndrome leading to the diagnosis of seronegative neuromyelitis optica spectrum disorder that was dramatically improved under biotin. We carry on a review of the literature of the previously reported pediatric cases with an opticospinal syndrome revealing biotinidase deficiency.