Paroxysmal vertigo as the presenting symptom of Fabry disease

Abstract

Abstract-The authors propose "decreased-dose-intensity" PCV (procarbazine, lomustine [CCNU], and vincristine) chemotherapy for Asian patients with oligodendroglial tumors. In this study, all seven patients with oligodendroglioma (OD) and eight with anaplastic oligodendroglioma (AO) had objective responses or stable disease. Median progression-free survival was greater than 29 months (OD) and 36.5 months or greater (AO); 86% of patients with OD and 63% with AO remain progression-free. Twenty-four Common Toxicity Criteria Grade 3/4 adverse events were noted. NEUROLOGY 2006;66:247-249 A.U. Ty, MD; S.J. See, MD; J.P. Rao, MD; J.B.K. Khoo, MD, FRCR; and M.C. Wong, FRCP Oligodendroglial tumors are among the most chemosensitive of human solid malignancies. In terms of cost and availability, PCV (procarbazine, lomustine [CCNU], and vincristine) chemotherapy is the most viable chemotherapeutic option for patients with oligodendroglial tumors in Asia. PCV-associated toxicity, particularly cumulative myelosuppression, is well described among white patients.1,2 There are no reports in the published literature describing Asian oligodendroglial PCV chemotherapy outcome. In our experience, most Asian patients cannot tolerate the toxicity associated with the Western "standard" PCV dose. We developed a "decreased-dose-intensity" PCV regimen for our Asian patients, and this study reports our experience. All patients underwent MRI scanning within 14 days before initiation of chemotherapy, after the second or third cycle, at the end of chemotherapy, when clinical progression was suspected, and at least 6 monthly follow-up intervals. Tumor size was measured via the product of the two largest perpendicular diameters. Metastasis along the ventricular lining or cortical surface was considered evidence of leptomeningeal disease (LMD). Postgadolinium T1-weighted images were used to measure enhancing tumors, and T2-weighted or fluid-attenuated inversion recovery images were used for non-contrast-enhancing tumors.Conventional measures of response such as complete response (CR), partial response (PR), stable disease, and progressive disease (PD) were used. 3 The proportion of [CR ϩ PR] constituted the objective response rate. Progression-free survival (PFS) was the period from initiation of chemotherapy to disease progression or death. Grade 3/4 Common Toxicity Criteria (NCI-CTC version 2.0) adverse events (AEs) were noted.Results. Treatment outcome and PFS data are summarized in table 1. No patient had development of PD while undergoing chemotherapy. Five patients with OD were given up-front PCV because they all had clinically symptomatic, radiologically extensive, surgically unresectable, and thus "aggressive" low-grade ODs. We observed early (after 2 or 3 cycles of PCV) objective responses in 57% of patients with OD and 13% of patients with AO. Among patients with OD, median PFS was greater than 29 months (range of Ͼ 23 to Ͼ 40 months). Six of seven patients (86%) remain progression free, whereas one patient (13%) had development of d...

Neuroimaging in Fabry disease: current knowledge and future directions

“…Anomalies of the vasculature wall of the posterior circulation in FD are usually asymptomatic [ 50 ]. Occasionally, posterior circulation dilatation can be also responsible for compression of anterior pons and cerebello-pontine angle neuro-vascular structures, causing symptoms such as auditory and vestibular dysfunction, trigeminal neuralgia or headache [ 51 , 52 ].…”

The Central Nervous System Involvement in Fabry Disease

“…Vertebrobasilar dolichoectasia (VBD) is a vascular condition that may cause severe morbidity due to ischemic or compressive dysfunction in the posterior fossa (ie, paroxysmal vertigo due to the compression of the VIII cranial nerve has been reported in a patient with FD). 34 Vertebrobasilar dolichoectasia may not be readily recognized by many neurologists or radiologists owing to the ''normal'' variation in the tortuosity or diameter of the vertebral and basilar arteries in healthy individuals. The study of dolichoectasia has been hampered by the lack of standardized diagnostic criteria.…”

Fabry’s disease: Neurological disorders

“…Возникают долихоэктазии церебральных сосудов, особенно в вертебрально-базилярной системе, что сопровожда-ется инсультами и ТИА [11,16]. Эктазия церебральных сосудов может быть настолько выраженной, что приводит к сдавлению желудочков головного мозга с развитием гидроцефалии и ком-прессионному поражению краниальных нервов [8,17]. Геморра-гические инсульты при БФ возникают крайне редко [15].…”

“…Пациенты безуспешно лечатся по поводу «синдрома хронической усталости», «хронической боли» или «ревматологи-ческого заболевания», «пароксизмального головокружения» [8,17,19]. Среди редких неврологических проявлений БФ следует упомянуть возникновение длительного эпизода амнезии, напо-минающего транзиторную глобальную амнезию [13] и паркинсо-низма [20].…”