Case report of Wegener's granulomatosis presenting with multiple cranial nerve palsy and hypertrophic cranial pachymeningitis]

We report a 41-year-old man whose initial neurological symptoms are atypical of Wegener's granulomatosis. The patient was admitted because he developed left ocular pain, headache, bilateral visual loss and left abducens nerve palsy. He was initially diagnosed with optic neuritis at ophthalmological department and steroid therapy was started. Although steroid therapy led to rapid recovery of visual acuity and eye movement, he was readmitted for seizure. Two weeks later, a second seizure attack occurred, followed by palsy of the left side of cranial nerves II, III, IV, V and VI. Brain MRI showed focal thickening and enhancement of the dura mater over left frontal lobe, leading to a new presumptive diagnosis of idiopathic hypertrophic cranial pachymeningitis. Steroid therapy was resumed and the symptoms improved rapidly. As right hemiparesis developed during the clinical course, another brain MRI was obtained. T2-weighted image showed a high intensity area in the left portion of the pons. 14 months later, recurrent epistaxis suggestive of Wegener's granulomatosis appeared. A subsequent nasopharyngeal mucosa biopsy revealed a necrotizing granulomatous inflammation. A significant elevation of PR-3 ANCA was also noted. A definitive diagnosis of Wegener's granulomatosis was established. The initial presentation of this case was of multiple cranial neuropathies with no superior respiratory tract symptoms, which are typical of early stage Wegener's granulomatosis. In patients with various central nervous system symptoms and MRI evidence of hypertrophic cranial pachymeningitis, a thorough clinical workup of vasculitis syndrome including Wegener's granulomatosis should be considered.     

Cerebral demyelination in Wegener's granulomatosis

A 38-year-old woman with a history of a granulomatous lesion of the nose, developed blurred vision, ataxic gait, and spastic tetraparesis. The presence of demyelination on the brain MRI led to the diagnosis of cerebral demyelination associated with Wegener's granulomatosis. Pulse cyclophosphamide administration resulted in some clinical of improvement of her condition. Demyelinating lesions seen in Wegener's have been ascribed to multiple sclerosis, but in this case, they are much more reminiscent of disseminated encephalomyelitis (DEM). The immunological challenge of the underlying disease, may, in the genetically susceptible person, presumably trigger the appearance of MS lesions. Wegener's granulomatosis must be considered in the differential diagnosis of MS.     

Risk of recurrent intracerebral hemorrhages

Intracerebral hemorrhage (ICH) occurs in about 10%-15% of all strokes, and hypertension and cerebral amyloid angiopathy (CAA) are the main underlying causes. There is often controversy regarding surgical evacuation especially in elderly patients. Follow-up of these patients and regulation of hypertension is important to prevent re-bleeding. The number of recurrent hematomas will increase with time of follow-up. We reviewed 968 patients with an ICH treated in our Department and 48 patients with recurrent hemorrhages (4.9%). The mean interval between the first and the second hemorrhage was three years (one month to 10 years). Clinical outcome after a second hemorrhage was severe and only 50% of patients were operated on the second hemorrhage compared to 77% (37/48) of patients who were operated on the first hemorrhage.     

Risk of recurrent intracerebral hemorrhages

Abstract

Intracerebral hemorrhage (ICH) occurs in about 10%-15% of all strokes, and hypertension and cerebral amyloid angiopathy (CAA) are the main underlying causes. There is often controversy regarding surgical evacuation especially in elderly patients. Follow-up of these patients and regulation of hypertension is important to prevent re-bleeding. The number of recurrent hematomas will increase with time of follow-up. We reviewed 968 patients with an ICH treated in our Department and 48 patients with recurrent hemorrhages (4.9%). The mean interval between the first and the second hemorrhage was three years (one month to 10 years). Clinical outcome after a second hemorrhage was severe and only 50% of patients were operated on the second hemorrhage compared to 77% (37/48) of patients who were operated on the first hemorrhage.     

Acute psychiatric symptomatology in Wegener's granulomatosis

A 69-year-old man presented with a manic episode but in fact was found to be suffering from Wegener's granulomatosis, a systemic disease that can attack the central nervous system. This case study demonstrates that in rare cases acute psychiatric symptoms can develop in a patient suffering from Wegener's granulomatosis, that need to be treated in the proper way. The purpose of the discussion is twofold: to draw attention to the rarity of the case and to stress the need for a multidisciplinary approach to systematic diseases.     

Fatal neurologic involvement in pediatric Wegener's granulomatosis

Wegener's granulomatosis is a potentially life-threatening vasculitis with widely variable presentation. Only three pediatric cases with severe central nervous system involvement are reported in the literature. Early fatal outcome as described here is exceptional. This report describes a 13-year-old female with typical skin lesions, proteinuria, and renal failure initially misdiagnosed as a Schoenlein-Henoch purpura. A kidney biopsy revealed severe extracapillary proliferation in 70% of the analyzed glomeruli but no granuloma. In spite of methylprednisolone pulse therapy and oral high-dose prednisone, end-stage renal failure was reached 4 months later, necessitating peritoneal dialysis. Three months later she presented with pulmonary hemorrhage and positive antineutrophil cytoplasmic antibodies suggesting Wegener's granulomatosis. This episode was controlled by methylprednisolone pulses. Seven months later she presented generalized seizures and coma, suggesting central nervous system involvement confirmed by magnetic resonance imaging. Methylprednisolone pulses and intravenous immunoglobulins led to neurologic improvement. Oral methotrexate was then introduced for long-term disease control. Another severe relapse of central nervous system vasculitis did not respond to any applied therapies and led to death 16 months after initial symptoms. This case emphasizes the need for activity scores to identify patients at risk for progressive systemic vasculitis requiring early and long-term aggressive immunosuppressive therapy.     

Delusion of parasitosis due to Wegener's granulomatosis

A delusion of parasitosis can be idiopathic or due to different somatic or psychiatric disorders. We report on a 48-year-old man who suffered from imagined animals in his nose. A somatic cause could not be found so that we diagnosed an idiopathic delusion of parasitosis. Later, Wegener's granulomatosis, a rare form of autoimmune induced vasculitis, was diagnosed. It was treated successfully with immunosuppressiva. The psychotic symptoms which remitted under risperidone did not return after finishing the neuroleptic medication.     

Seizures and antiepileptic drugs in patients with spontaneous intracerebral hemorrhages

PurposePatients with intracerebral hemorrhage (ICH) are often initiated on antiepileptic drugs without a clear indication. We compared the percentage of patients with spontaneous ICH who had seizures at onset or during hospitalization, and examined empiric use of antiepileptic drugs (AEDs) in these patients in 2 cohorts 10 years apart.MethodsUsing a clinical data registry at a tertiary care adult hospital, we retrospectively selected admissions for spontaneous ICH between 1/1/99–12/31/00 (Cohort A, n = 30) and 1/1/09–12/31/10 (Cohort B, n = 108). Clinical, neurophysiological and radiological data were collected in both cohorts.ResultsIn Cohorts A and B respectively, AEDs were started in 53.3% and 50.0%, and continued on discharge in 50.0% and 20.4% of patients; 86.6% and 59.1% of patients discharged on AEDs did not have a clinical/electrographic seizure or epileptiform EEG findings. Seizures occurred in 6.6% and 13.0% in Cohorts A and B respectively. The presence of a seizure at presentation (p = 0.01) and during hospitalization (p = 0.02) were predictors for continuing AED on discharge.ConclusionIn both cohorts, a significant number of patients were discharged on AEDs without a clear indication, though there is a change in practice between the two cohorts.     

自发性多部位脑出血临床研究

目的总结自发性多部位脑出血(MICH)的临床特征。方法分别采取保守治疗、穿刺引流血肿清除术、血肿清除术和(或)去骨瓣减压术治疗30例自发性多部位脑出血和600例单一部位脑出血(SICH)患者;采用改良Rankin量表评价发病后3个月预后。结果与SICH组相比,MICH组患者高血压5年(P=0.008)、糖尿病(P=0.024)、高胆固醇血症(P=0.050)和缺血性卒中(P=0.026)发生率均较高,发病后平均动脉压升高(P=0.002)且多伴有肢体活动障碍(P=0.000),基底节和丘脑为出血好发部位(P=0.001)。随访至发病后3个月,MICH组患者预后不良(P=0.006)。结论高血压5年、糖尿病、高胆固醇血症和缺血性卒中是诱发多部位脑出血的病理生理学基础,患者临床症状严重、远期生活质量差。     

Distinguishing intracerebral hemorrhages caused by arteriovenous malformations

BACKGROUND: There is a shortage of data addressing the clinical characteristics of patients with arteriovenous malformations (AVMs) who present with intracerebral hemorrhages (ICH). METHODS: A retrospective cohort study of members of a large, pre-paid health care program was conducted to identify factors that distinguish ICH secondary to cerebral AVMs from all other causes. Univariate and multivariate analysis was performed using Student's t test, Wilcoxon rank-sum test, and logistic regression. RESULTS: Patients with an underlying AVM were younger and more likely to be female, non-smokers with lower blood pressures, lower cholesterol, and lower white blood cell counts on presentation. CONCLUSIONS: These clinical characteristics may be useful in defining potential risk factors in future prospective studies as well as targeting candidates for additional imaging studies after ICH with no apparent etiology.     

A diagnostic approach to multiple simultaneous intracerebral hemorrhages

Multiple simultaneous intracerebral hemorrhages are rare and varied in etiology. In the absence of known risk factors or obvious underlying disease, determining the cause may be problematic. We outline a diagnostic approach to multiple simultaneous intracerebral hemorrhages in the context of three case studies. We conclude that when there are no apparent risk factors, neuroimaging and identification of underlying diseases are central to determining the cause of multiple simultaneous intracerebral hemorrhage.