Case Reports in Neurology最新文献

Central nervous system vasculitis (CNSV) is an uncommon and poorly understood form of vasculitis. Early recognition is important because medical treatment might improve the outcome. However, randomized clinical trials on CNSV treatment do not exist. Endovascular treatment has been reported in few cases, but no data exist for intracranial stenting. We report 2 cases of patients with suspected CNSV and recurrent clinical episodes, treated with intracranial stenting. A 48-year-old man had relapsing episodes of right hemiparesis. Neuroradiological exams showed severe left carotid terminus stenosis. Despite immunosuppressive therapy, neuroradiological follow-up exams showed a worsening of the aforementioned stenosis with many transient episodes of weakness in the right limbs and aphasia. A 64-year-old woman had a sudden onset of dysarthria and transient aphasia. Neuroradiological exams showed a severe arterial stenosis involving the origin of left anterior cerebral artery and middle cerebral artery (MCA). Despite dual antiplatelet therapy, she presented an acute onset of severe aphasia, due to an occlusion of the left carotid terminus and proximal MCA. In both cases, endovascular procedure and intracranial stenting was performed, with marked improvement of cerebral blood flow. No more clinical episodes were reported. Intracranial stenting may be a valid therapeutic option in selected patients with CNSV and involvement of medium or large size vessels with clinical worsening despite best medical treatment.

This case report highlights a possible consequence of damage to the left frontoinsular region. A 53-year-old woman with chronic obesity and headaches presented with seizure, leading to the discovery and resection of a large sphenoid wing meningioma. Postoperative brain imaging revealed loss of the left frontoinsular cortex and portions of the underlying white matter, claustrum, and striatum. Throughout her adult life, this patient had tried and failed to lose weight, but after surgery, she no longer desired to eat large meals, and without effort, her body mass index decreased from 38.6 (85th percentile) to 24.9 (25th percentile). Combined with previous research implicating the insular cortex in interoception, appetite, and drug-related urges, her reduced hunger and effortless weight loss after resection of the left frontoinsular cortex suggest that this region of the human brain may play a role in hunger-related urges that contribute to overeating.

Posterior ischemic optic neuropathy (PION), a relatively rare condition, is diagnosed primarily based on the clinical presentation of sudden visual impairment, an optic nerve-related visual field defect, and an initial normal optic disc that corresponds to its pathology of acute ischemia. Among its etiologies, nonarteritic PION is one of the most common causes. Studies on cases of PION associated with herpes zoster ophthalmicus (HZO) are limited, and the diagnosis was made based on the appearance of visual symptoms shortly following rashes. We describe a 64-year-old Asian woman with sudden painless visual loss in the upper half visual field of the left eye 6 weeks after ipsilateral HZO. Within a week, her left vision progressed to total visual loss. Initial examination revealed a near-total visual defect and a normal appearance of the optic disc in the left eye. Laboratory and imaging studies excluded the compressive, infiltrative, or inflammatory etiologies of the left optic nerve. Considering the temporal relationship between the skin rash and visual loss, HZO was the most likely cause of the nonarteritic PION. The patient was given a short course of oral valaciclovir and aspirin. At 6 weeks after the visual loss, an examination revealed stationary visual acuity and visual field defect in the left eye with a pale optic disc, and a retinal nerve fiber loss in the left eye. Compared with previous studies, our case demonstrated a delayed presentation of nonarteritic PION following HZO and broadened the scope of herpes zoster optic neuropathy.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) affects multiple body systems, including the nervous system. Cerebrovascular accidents can also occur. Patients with comorbid illnesses have severe manifestations and poor outcomes. Despite the proper mechanism of SARS-CoV-2 infection-associated stroke having not yet been settled, various possible mechanisms have been hypothesized. One possibility is that the virus causes endothelial dysfunction and immune-mediated injury. Another possibility is that the trans-neuronal spread of the virus affects brain tissue. In addition, hypercoagulability caused by SARS-CoV-2 infection could lead to a stroke. A virus-induced dysfunction of the renin-angiotensin system could also lead to a stroke. The immune response and vasculitis resulting from SARS-CoV-2 infection are also possible causes via a cytokine storm, immune dysfunction, and various inflammatory responses. SARS-CoV-2 infection may affect calcitonin gene-related peptides and cerebral blood flow and may lead to stroke. Finally, SARS-CoV-2 may cause hemorrhagic strokes via mechanisms stimulated by its interaction with angiotensin-converting enzyme 2 (ACE2), leading to arterial wall damage and blood pressure changes. In this article, we will present seven cases of stroke-associated SARS-CoV-2 infection.

Multiple case series have demonstrated the emergence of small fiber neuropathy following acute coronavirus disease 2019 (COVID-19) infections. Further, one large case supports that the COVID-19 vaccine has been reported to result in small fiber neuropathy. We report a case of a patient with confirmed small fiber neuropathy post-COVID-19 vaccination with positive FGFR3 antibodies. The effect of intravenous immunoglobulin (IVIG) has been recently explored for treatment of presumed autoimmune small fiber neuropathy. To our knowledge, this is the first published case report of COVID vaccination-induced FGFR3-associated small fiber neuropathy improving in the context of IVIG administration as demonstrated by normalization of small fiber density measured by skin biopsy accompanied by marked improvement in the patient's symptoms.

Pleomorphic xanthoastrocytoma (PXA) is a rare glioma. It accounts for less than 1% of all astrocytomas. About 98% of PXAs originate supratentorially with the temporal lobe being the most common location. Cases of infratentorial PXAs are rarely reported in the medical literature. The tumor presents with a wide variation of symptoms based on the neuroanatomy involved with the location and size of the tumor, with seizures being the most reported symptom. The diagnosis depends on histological and clinical features along with radiologic features. We searched the keywords "Pleomorphic xanthastrocytoma, PXA, cerebellum, infratentorium, astrocytoma, gliomas" in the PubMed database; from 1979 to the current date, 28 cases were found in the medical literature featuring PXA in the infratentorium. We present the 29th case in the literature and the first in Syria. Our patient had a lesion in the right cerebellum and presented with a history of intermittent headache for 5 months followed by progressive gait disturbances and blurry vision and was misdiagnosed at the time of presentation with a high-grade glioma which is a common confusion because of the histological and clinical similarities. The patient underwent a suboccipital craniotomy, and adjuvant therapy with a combination of radiotherapy and chemotherapy with temozolomide was initiated at first. Then, the patient presented with a relapse of symptoms and went through another surgery where frozen sections suggested the diagnosis of PXA; further histopathological and immunohistochemical studies confirmed the diagnosis. Alongside highlighting the diagnostic challenge of this rare tumor, we did a brief review of the literature.

Diffuse midline gliomas are a new entity in the WHO Classification of Tumors of the Central Nervous System, corresponding to grade 4 gliomas. The diagnostic pathognomonic feature is the presence of a H3K27M mutation. Although mainly seen in children, cases in adults have also been reported. The symptoms are highly variable and usually dependent on the location and extent of spinal cord compression.

Hyper-/hypoglycemic states are rare but well-established causes of hyperkinetic movements, including chorea and ballismus, usually associated with brain lesions in the basal ganglia. We report a case of hemichorea-hemiballismus (HCHB) syndrome that developed after a severe hypoglycemic episode in a 71-year-old man with poorly controlled type 2 diabetes mellitus. Uncommonly, brain MRI showed contralateral cortical-subcortical T2 and T2-FLAIR-hyperintense frontoparietal lesions, with cingulate gyrus involved, while the basal ganglia were unaffected. In patients with hypoglycemic encephalopathy associated with cortical lesions, the long-term prognosis is usually poor. Nevertheless, in our patient, the dyskinesias and the cerebral lesions progressively regressed by achieving good glycemic control. After four and 12 months, the patient's neurological examination was normal. To our knowledge, this is the first evidence of hypoglycemic etiology of cortical HCHB syndrome, supporting recent theories that cortical circuitries may independently contribute to the pathogenesis of chorea and ballismus. This is also the first report of cingulate gyrus involvement in hypoglycemic encephalopathy. Finally, this case may indicate that a subset of patients with cortical lesions due to hypoglycemia could present a good clinical outcome, likely depending on the size of the lesions and the duration and severity of the hypoglycemic episode.

Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease with various neurological manifestations, including tremor. Here, we report a case involving a 68-year-old man with an 8-year history of tremor in his right arm. Subsequently, examination revealed that the patient was suffering from a low-frequency, high-amplitude, and posture-induced proximal arm tremor elicited by sustained arm abduction with flexed elbows (wing-beating tremor), which was partially improved by zonisamide treatment. Abnormal expansion of GGC repeats in the NOTCH2NLC gene confirmed the diagnosis of NIID. This case highlights the fact that unilateral wing-beating tremor can be a manifestation of NIID. Zonisamide may be effective for controlling tremors associated with NIID.

Hypertrophic pachymeningitis (HP) is a rare immune-mediated disease characterized by thickening of the dura mater with consecutive cranial neuropathy. While HP is usually treated with systemic immunotherapies, response to therapy is variable and may be limited by insufficient drug concentrations in the brain. We report on a 57-year-old patient with HP manifesting with vision and hearing loss who had sustained clinical progression despite various systemic immunotherapies. Intraventricular chemotherapy with methotrexate, cytarabine, and dexamethasone was initiated. We present clinical, imaging and cerebrospinal fluid (CSF) findings, including cytokine levels before and after intraventricular treatment: rapid decrease of cell count, lactate and profibrotic cytokine levels in the CSF following intraventricular chemotherapy was paralleled by a mild reduction of dura thickness in MRI. The already severely impaired visual acuity and hearing loss did not progress further. Treatment was complicated by exacerbation of previously subtle psychiatric symptoms. Follow-up was terminated after 6 months as the patient suffered from a fatal ischemic stroke. Autopsy revealed neurosarcoidosis as the underlying cause of HP. This case report suggests that intrathecal chemotherapy can reduce the inflammatory milieu in the CNS and should be considered for treatment-refractory HP before irreversible damage of cranial nerves has occurred.