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Behçet disease (BD) is a multisystemic relapsing autoimmune vascular disorder. It is clinically characterized by recurrent oral ulcers, genital ulcers, eye, and skin manifestations. Development of neurological symptoms in BD cases is rare and occurs several years after the initial diagnosis. We describe a rare case of a 39-year-old Saudi male who presented with isolated neurological manifestations as the first sign of BD. The patient had recurrent strokes, both ischemic and hemorrhagic, over an 11-month period before developing typical BD features. A thorough investigation excluded other potential etiologies of his neurological disorders. Imaging showed multiple brainstem lesions compatible with parenchymal neuro-BD (NBD). The patient was positive for HLA-B51, a genetic marker linked to BD, but had a negative pathergy test. Treatment with corticosteroids and infliximab resulted in symptom improvement. The diagnosis of NBD requires a comprehensive clinical, imaging, and laboratory assessment to rule out other possible causes. This case demonstrates the need to include NBD in the differential diagnosis of young patients with unexplained neurological manifestations, especially if they are followed by an onset of BD features. Treatment with corticosteroids and biologic agents can achieve favorable outcomes. NBD can present with isolated neurological symptoms, emphasizing the need for a high level of suspicion and a multidisciplinary approach for accurate diagnosis and effective management.
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Introduction: Herpes simplex virus (HSV) is associated with one of the lethal diseases, Herpes simplex encephalitis (HSE). Diagnosis is confirmed using MRI and CT scan imaging techniques and more sensitive DNA PCR from cerebrospinal fluid analysis and brain biopsy.Case presentation: However, after four days, the patient's HSE diagnosis was confirmed by the detection of herpes simplex virus type 1 (HSV1) via polymerase chain reaction (PCR) testing. This case highlights the importance of utilizing multiple diagnostic aids and not solely relying on initial test results, as infections may not appear in CSF analysis or MRI scans initially. Furthermore, this case also emphasizes the necessity of initiating empirical treatment based on clinical signs and symptoms, even in cases where diagnostic tests initially appear negative. Prompt and efficient diagnosis and treatment are crucial in managing HSE and preventing long-term neurological damage.Conclusion: This case of HSE underscores the significance of a multifaceted diagnostic approach and timely intervention in managing this potentially severe and life-threatening condition. As mentioned, sometimes the infection does not appear in CSF analysis initially, nor does its effects appear in MRI. HSV PCR remains the golden test to confirm the diagnosis of HSE.
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Perampanel (Fycompa) is a glutamate receptor antagonist known to be a safe, effective, and well-tolerated medication; nevertheless, adverse effects are possible. This case report aims to raise the suspicion of perampanel-induced thrombocytopenia and discuss its possible pathways implicated. Here, we present the case of a 66-year-old female patient with a generalized tonic-clonic seizure initially managed with levetiracetam, valproic acid, and lacosamide; however, the patient continued to have seizures clinically as well as on the electroencephalogram. The patient was initiated on 2 mg of perampanel and reached up to 12 mg within a week, after which the seizure was controlled. Nevertheless, after perampanel initiation, a gradual platelet count reduction was observed. Upon withdrawal of perampanel, the platelet count dramatically improved reaching up to her baseline. Although perampanel is known to be a safe medication, a hematological complication such as thrombocytopenia is possible. The exact mechanism remains unclear. Further studies are required to understand the association between thrombocytopenia and perampanel to identify high-risk populations and prevent this condition sequentially.
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Multiple sclerosis (MS) is a chronic disease of the central nervous system (CNS). It has many types, which include tumefactive multiple sclerosis (TMS), one of the most uncommon types. We present the case of a 36-year-old woman who presented with right-sided numbness of the body. Magnetic resonance imaging (MRI) of the brain revealed a large mass (3 cm × 2.5 cm) in the deep white matter of the right frontal lobe along with smaller lesions of variable sizes. After considering the MRI features, the CSF results, and the improvement of the symptoms with a high dose of steroids, the diagnosis of tumefactive multiple sclerosis was made. A biopsy was not done on our patient as the symptoms resolved after treatment, although sometimes it is necessary for diagnosing tumefactive multiple sclerosis to rule out tumors or abscesses. The current study described the clinical presentation, the role of imaging, the differential diagnosis, and the treatment options. This case report aimed to report a rare presentation of TMS, which highlights the importance of differentiating TMS from other space-occupying lesions for prompt and proper management.
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Coronavirus disease 2019 (COVID-19) pandemic is caused by the severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) and since the outbreak, many neurological features and syndromes are reported with this multi-organ viral infection. Lance-Adams syndrome (LAS) also referred to as chronic post hypoxic myoclonus is defined as action myoclonus which can occur as generalized, focal, or multifocal repeated myoclonic motor movements which involve the face, trunk, or extremities and it is one of the neurological complications that are related to COVID-19 infection. LAS is reported as a delayed complication of cardiac arrest, which causes cerebral hypoxia leading to myoclonus. We report a case of a 58-year-old male patient diagnosed as a case of LAS secondary to hypoxia occurring because of COVID-19 without cardiac arrest and to the best of our knowledge it is the second case reported with this similar mechanism. Moreover, we discuss the possible pathophysiological relationship between LAS and COVID-19 and various treatment strategies. Eventually, we review the related articles in the literature regarding the LAS and various types of myoclonus associated with COVID-19 infection.
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Neuromyelitis optica (NMO) is an autoimmune demyelinating disorder of the central nervous system which is characterized by attacks of optic neuritis and transverse myelitis. An association between NMO and intracerebral hemorrhage (ICH) has been rarely recognized, having been reported only 3 times before. Here we report on a patient with NMO who eventually developed subarachnoid hemorrhage, in order to emphasize that the association between NMO and ICH is mostly not incidental and that the pathological basis for this association should be investigated thoroughly.
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An intracranial abscess caused by Proteus mirabilis is rarely reported in adults. A 17-year-old girl presented with generalized tonic-clonic seizure, high-grade fever, headache, and vomiting with a history of slowly progressing apathy, clumsiness, and disorientation. She had meningeal signs and altered sensorium with a Glasgow Coma Scale of 10. The laboratory analysis revealed leukocytosis and elevated erythrocyte sedimentation rate. Brain computed tomography (CT) revealed a cystic lesion in the left temporal lobe with perilesional edema and a slight midline shift. She was commenced on empiric ceftriaxone, amikacin, and metronidazole. The non-foul smelling pus was drained through a craniotomy, and pus culture showed P. mirabilis. Culture sensitivity revealed extended-spectrum B-lactamase production, and she was commenced on intravenous carbapenem in addition to existing drugs. A repeat CT revealed a significant reduction in abscess size, and improvement in her condition was observed. On her recent follow-up visit, she was doing well.