A 14-year-old boy was admitted to the hospital due to a single episode of afebrile seizure and four hours of impaired consciousness. Three months prior to admission, he had a history of bilateral uveitis. Cerebrospinal fluid analysis revealed a mild elevation in white blood cell count. Cranial magnetic resonance imaging and contrast-enhanced scans showed multiple abnormal signals in both cerebral hemispheres, with punctate and nodular enhancement. Susceptibility-weighted imaging revealed multiple punctate hemorrhages within lesions in the bilateral frontal and left parietal lobes, suggestive of vasculitis. Brain biopsy demonstrated inflammatory granulomatous lesions. No secondary causes were identified, and the final diagnosis was granulomatous primary central nervous system vasculitis. The patient's condition improved after treatment with methylprednisolone sodium succinate and mycophenolate mofetil. This report describes a rare case of granulomatous central nervous system vasculitis in a child and provides valuable insights for the diagnosis and treatment of this disease.
Humans ; Male ; Vasculitis, Central Nervous System/diagnosis* ; Adolescent ; Magnetic Resonance Imaging ; Granuloma/diagnosis*

Primary central nervous system vasculitis (PCNSV) is rare, and the diagnosis is difficult to make because of its variable radiologic expressions. Early corticosteroid therapy often is effective. Herein we report the case of a 56-year-old man who had a well-enhanced cystic mass with severe edema in the right frontal lobe, which was initially felt to be a malignancy. Histologic examination of tissue removed at craniotomy revealed that it was a PCNSV. Despite early administration of corticosteroids, a new lesion developed within 3 days. The lesions responded to treatment with cyclophosphamide and corticosteroid.
Adrenal Cortex Hormones ; Craniotomy ; Cyclophosphamide ; Diagnosis ; Edema ; Frontal Lobe ; Glucocorticoids ; Humans ; Middle Aged ; Vasculitis ; Vasculitis, Central Nervous System

Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Approximately 15% of Degos disease have a benign course limited to the skin and no history of gastrointestinal or central nervous system (CNS) involvement. A 29-year-old female with history of systemic lupus erythematosus (SLE) presented with a 2-year history of asymptomatic lesions on the dorsum of all fingers and both knees. The patient had only skin lesions and no gastrointestinal or CNS vasculitis symptoms. Her skin lesions were umbilicated, atrophic porcelain-white lesions with a rim of erythema. On the basis of clinical, histologic, and laboratory findings, a diagnosis of Degos-like lesions associated with SLE was made. The patient had been treated for SLE for 7 years. Her treatment regimen was maintained over a 2 month follow-up period, and the skin lesions improved slightly with no development of new lesions.
Adult ; Autoimmune Diseases ; Central Nervous System ; Diagnosis ; Erythema ; Female ; Fingers ; Follow-Up Studies ; Humans ; Infarction ; Knee ; Lupus Erythematosus, Systemic* ; Malignant Atrophic Papulosis ; Skin ; Vasculitis ; Vasculitis, Central Nervous System

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.
Asthma ; Brain ; Caudate Nucleus ; Cerebral Infarction ; Cerebrospinal Fluid ; Churg-Strauss Syndrome ; Cyclophosphamide ; Diagnosis ; Diffusion Magnetic Resonance Imaging ; Eosinophilia ; Eosinophils* ; Extremities ; Fever ; Gadolinium ; Gait ; Granulomatosis with Polyangiitis* ; Guillain-Barre Syndrome ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Muscle Weakness ; Neural Conduction ; Peripheral Nervous System Diseases ; Polyneuropathies* ; Sensation ; Vasculitis ; Vasculitis, Central Nervous System*

PURPOSE: To report a rare case of Aspergillus endophthalmitis as a preceding symptom of central nervous system (CNS) lymphoma. CASE SUMMARY: A 66-year-old female was admitted to our clinic with mental change for 3 days. Seven months earlier, she had been diagnosed with retinal vasculitis in an ophthalmology clinic because of blurred vision in both eyes and was administered steroid therapy. Three months earlier, because of progressive symptoms, vitreous fluid culture had been performed and showed Aspergillus endophthalmitis. She was treated with intravitreous voriconazole injection and oral voriconazole. Initial brain magnetic resonance imaging (MRI) and cerebrospinal fluid study was normal. Two months later, a second MRI showed multiple enhancing lesions, which were aggravated on the third MRI at admission to our clinic. Although brain biopsy was not performed due to the poor condition of the patient, CNS lymphoma was suspected based on the neuroimaging. After steroid pulse therapy and whole brain radiation, follow-up neurologic examination showed improved mental state, and follow-up MRI showed remarkable shrinkage of multiple lesions. CONCLUSIONS: As Aspergillus endophthalmitis is an opportunistic infection in those with an immune-compromised state and the orbit is near the central nervous system, the clinician should be alert to concomitant disorders in CNS. For a prompt and accurate diagnosis of CNS disorder, early evaluation of neurologic symptoms beyond symptoms of endophthalmitis and neuroimaging is essential.
Aged ; Aspergillus* ; Biopsy ; Brain ; Central Nervous System* ; Cerebrospinal Fluid ; Diagnosis ; Endophthalmitis* ; Female ; Follow-Up Studies ; Humans ; Lymphoma* ; Magnetic Resonance Imaging ; Neuroimaging ; Neurologic Examination ; Neurologic Manifestations ; Ophthalmology ; Opportunistic Infections ; Orbit ; Retinal Vasculitis

BACKGROUND AND PURPOSE: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease that causes acute visual symptoms with characteristic ophthalmoscopic findings. Neurological complications are rarely reported in the literature. Here we report two new cases of APMPPE that presented with neurological manifestations, one of which was associated with peripheral neuropathy, which has not been described before. METHODS: A retrospective database review of all patients with a diagnosis of APMPPE was performed. Clinical, ophthalmological, and neurological data were analyzed, and only cases of APMPPE with neurological complications were included. A literature review of several databases was also performed, and previous case reports were reviewed and analyzed in detail. RESULTS: In total, 56 cases of APMPPE-associated neurological complications were included in the analyses: 54 from the literature and 2 from our own practice. The most common complication was cerebral vasculitis, which affected 28 patients (50%), followed by headaches in 15 patients (26.8%). The other complications include sixth-cranial-nerve palsy, transient hearing loss, meningoencephalitis, cavernous sinus thrombosis, and viral meningitis. CONCLUSIONS: This report adds to the literature of a novel association of APMPPE with peripheral neuropathy, and comprehensively reviews the neurological manifestations of this disease. A high level of suspicion should be applied when dealing with a case of APMPPE. We recommend applying detailed clinical neurological examinations and magnetic resonance imaging to APMPPE patients, and then early steroid treatment if the examination is positive or even suspicious. Early treatment with steroids and long-term treatment with immunosuppressive azathioprine with interval neurological evaluations will contribute positively to the outcomes and avoid fatal complications, namely strokes.
Adrenal Cortex Hormones ; Azathioprine ; Cavernous Sinus Thrombosis ; Diagnosis ; Headache ; Hearing Loss ; Humans ; Magnetic Resonance Imaging ; Meningitis, Viral ; Meningoencephalitis ; Neurologic Examination ; Neurologic Manifestations* ; Paralysis ; Peripheral Nervous System Diseases ; Retrospective Studies ; Steroids ; Stroke ; Vasculitis, Central Nervous System

Brucellosis is a multisystem disease with various clinical symptoms. Neurobrucellosis is a rare but serious manifestation of brucellosis. A 60-year-old man with a previous diagnosis of brucellar spondylitis presented with sudden onset of aphasia and numbness of the right upper extremity. Cerebral angiography showed diffuse narrowing and dilatation on the distal branches of both the anterior cerebral artery (ACA) and the left middle cerebral artery (MCA) which indicated cerebral vasculitis, and the patient's Brucella agglutinin titer was 1:1280. After combined antimicrobial and steroid therapy was started, the patient's condition improved significantly, and he was discharged after 1 month. Antimicrobial therapy was continued for 16 months on an outpatient basis, and the last Brucella agglutinin titer was 1:40. To our knowledge, this is the first case of relapsed neurobrucellosis with vasculitis in Korea to have been treated successfully.
Anterior Cerebral Artery ; Aphasia ; Brucella ; Brucellosis* ; Cerebral Angiography ; Diagnosis ; Dilatation ; Humans ; Hypesthesia ; Korea ; Middle Aged ; Middle Cerebral Artery ; Outpatients ; Spondylitis* ; Upper Extremity ; Vasculitis ; Vasculitis, Central Nervous System*

BACKGROUND: Primary angiitis of the central nervous system (PACNS) is a rare disorder and is often difficult to diagnose due to the lack of a confirmatory test. PACNS can generally be diagnosed based on typical angiographic findings. We describe herein a patient diagnosed with PACNS despite the presence of normal findings on conventional angiography. CASE REPORT: A 44-year-old man with a recent history of ischemic stroke in the right posterior cerebral artery territory developed acute-onset vertigo. Diffusion-weighted imaging revealed an acute infarction within the left posterior inferior cerebellar artery. His medical history was unremarkable except for hyperlipidemia; the initial examination revealed mild gait imbalance. During the 10 days of hospital admission, the patient experienced four recurrent ischemic strokes within the posterior circulation territory (occipital lobe, pons, and cerebellum). He was diagnosed with recurrent cerebral infarctions due to PACNS. The basilar artery exhibited no demonstrable luminal stenosis, but there were direct imaging signs of central nervous system angiitis including wall thickening and contrast enhancement. High-dose intravenous steroid therapy followed by oral prednisolone was administered. There was no further stroke recurrence and follow-up imaging of the arterial walls showed normalization of their characteristics. CONCLUSIONS: The present case emphasizes the importance of wall imaging in the diagnosis and treatment of PACNS.
Adult ; Angiography ; Arteries ; Basilar Artery ; Central Nervous System* ; Cerebral Infarction ; Constriction, Pathologic ; Diagnosis* ; Follow-Up Studies ; Gait ; Humans ; Hyperlipidemias ; Infarction ; Inflammation ; Magnetic Resonance Imaging* ; Phenobarbital ; Pons ; Posterior Cerebral Artery ; Prednisolone ; Recurrence ; Stroke ; Vasculitis* ; Vasculitis, Central Nervous System ; Vertigo

Primary angiitis of the central nervous system is a rare and difficult entity. Here we represented the clinical and pathological features of a patient with little response to steroid before definite diagnosis. The 50-year-old male had a fluctuating disease course for more than 3 years. He presented visual disorders, seizure, cognitive impairment, hypersomnia, unsteady gait, dysphasia, dysphagia, and incontinence. Magnetic resonance imaging showed multiple, supratentorial and infratentorial abnormal signals, while cerebrospinal fluid and cerebral angiography were normal. Magnetic resonance spectrum showed a decrease of N-acetyl-aspartate. Brain biopsy revealed nongranulomatous lymphatic vasculitis with reactive gliosis, cicatrization, demyelination and focal hemorrhages.
Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Vasculitis, Central Nervous System ; diagnosis

Churg-Strauss syndrome (CSS) is an uncommon disorder that is characterized by asthma or allergic rhinitis, fever, systemic vasculitis, extravascular granulomas and hypereosinophilia. Cutaneous manifestations of CSS are one of the most common extra-pulmonary findings, and they occur in approximately 60% of the cases. Other manifestations include pulmonary infiltrates and heart, kidney, gastrointestinal tract, peripheral and central nervous system involvement and the patients also complain of systemic symptoms, including malaise, fever, myalgia and weight loss. The diagnosis of CSS is difficult because the manifestations vary with the disease stage and delayed treatment can lead to fatal major organ damage. We report here on 3 cases of CSS that were first diagnosed by the dermatologic department and with consultation from other departments. So, we want to remind dermatologists that if the patients of asthma or other allergic diseases have purpuric skin lesion, then CSS should be included in the differential diagnosis.
Asthma ; Central Nervous System ; Churg-Strauss Syndrome ; Diagnosis, Differential ; Fever ; Gastrointestinal Tract ; Granuloma ; Heart ; Humans ; Kidney ; Rhinitis ; Rhinitis, Allergic, Perennial ; Skin ; Systemic Vasculitis ; Vasculitis ; Weight Loss