Case Report/Case Series
The term “parkinsonism” usually refers to a group of diseases, including movement disorders. It is a syndrome characterized by bradykinesia, rigidity, and tremors. Parkinsonism is easily linked to Parkinson disease, particularly in the elderly.1 However, some other factors and differential diagnoses should be considered. Although the direct cause and mechanisms of Parkinson disease remain unknown, it is now thought to be related to genetic and environmental factors and responsive to dopaminergic medications.2 In contrast, for the patients with parkinsonism, the prompt and accurate diagnosis of the reversible underlying conditions might be critical. Differential diagnosis of Parkinson disease and parkinsonism is complicated and difficult. The spectrum of parkinsonism includes neurodegenerative disorders, infectious diseases, cerebrovascular diseases, metabolic disorders, exposure to drugs and toxins, and structural brain lesions, etc.3
Dural arteriovenous fistula (DAVF) is a vascular malformation. A dural fistula is an abnormal crossover connection between 2 sides of the dural layer. Penetrating the dura mater, arachnoid mater, and pia mater, DAVF directly drains blood flow from the meningeal and extracranial arteries into the meningeal veins, pia veins, and/or dural sinuses.4 The true incidence and prevalence of DAVF is not known because patients with DAVF might completely be asymptomatic.5 In addition, DAVF might mimic other neurological conditions because of its location and the associated effects of space occupation and brain tissue perfusion.
We present a case of a patient with left transverse sinus DAVF with sigmoid thrombosis, cortical venous reflux, and congestion, that initially presented as parkinsonism.
A 57-year-old man without any history of head trauma or central nervous system infection visited the clinic because of his clumsy left hand. He was healthy, was not taking any medication, and had no known risk factors for cerebrovascular diseases. He complained that he currently could not perfectly play a Chinese violin as he used to.
During the examination, the patient was alert and fully oriented; however, decreased facial expressions were noted. His left forearm showed rigidity and bradykinesia, and it was difficult for the patient to smoothly perform rapid alternating movement testing. Parkinsonism and Parkinson disease were then suspected. Although his muscle strength of the bilateral limbs was symmetric and full, his complaints during the following visit about hearing a rhythmic bruit above his left ear, particularly when lying down, alerted the physician about the presence of vascular lesions.
Electroencephalography displayed slow waves over the left temporal area, and magnetic resonance imaging and angiography (MRI and MRA, respectively) showed engorged and tortuous vessels above the cerebellum, the deep gray matter, and the left temporal lobe (Fig. 1). Digital subtraction angiography (DSA) revealed early opacification of the left transverse sinus with occlusion of the sigmoid sinus. Reverse blood flow to the straight sinus and right transverse sinus and many engorged cortical veins were also noted (Fig. 2). The diagnosis of left transverse sinus DAVF with sigmoid thrombosis and cortical venous reflux was confirmed.
Transvenous embolization through the occluded sigmoid sinus was attempted but failed because the sigmoid thrombosis was too long to fully advance the catheter. Intervention from the opposite transverse sinus also failed because of a tortuous confluence of sinuses. Finally, the patient received a combined surgical and endovascular approach. The neurosurgeon punctured the left transverse sinus directly with an 18-G needle sheath through a burr hole in the left occipital bone. A microcatheter was inserted into the left transverse sinus via the sheath, and detachable coils were deployed in the distal sigmoid sinus posterior to the distal left transverse sinus. After embolization, only minimal residual fistula with slow flow remained.
One month later, the patient mentioned that the noise above his left ear had almost disappeared, and the movement of his left forearm and hand improved. The patient underwent a follow-up MRA and DSA, which showed a decreased volume of tortuous vessels. The previous fistula was completely occluded.
In this case, we demonstrated that DAVF might be listed as one of the differential diagnoses in patients with secondary parkinsonism. The timely diagnosis and definite treatment prevented prolonged useless treatment in this patient, as well as potential critical complications such as intracranial hemorrhage.5
In 1999, Matsuda et al6 reported on 3 patients (2 women and 1 man) with DAVF who had major symptoms of progressive dementia and parkinsonism, which may be the first case reported on the association of DAVF and parkinsonism. However, when searching on PubMed for DAVF and parkinsonism, only sporadic cases were found reporting the link between DAVF and parkinsonism in the last 2 decades.6–14 Almost all the reports were in Asia (6, 3, 1, 1, and 1 in Japan, China, South Korea, India, and Israel, respectively), and only one 79-year-old man was reported in North America,13 with DAVF in the traditional occidental area. These patients were predominantly male (only 3 female patients), with an age distribution from 40 to 79 years.
Previously, DAVF was thought to be congenital but is now considered an acquired disease associated with venous hypertension, thrombosis, and factors triggering angiogenesis.4 The presented symptoms of DAVF range from asymptomatic to emergent critical and might depend on the location, pattern of venous drainage, and vascular status at diagnosis. The symptoms might be varied and nonspecific. With the increased accessibility to computed tomography, MRI, and MRA, the diagnosis of DAVF may become more accurate and efficient.15,16
In this case, DAVF with retrograde flow caused venous congestion and ischemia above the right basal ganglia, internal capsule, thalamus, left cerebellum, and left temporal lobe, as well as a hemorrhage above the right basal ganglia and thalamus. These are likely the cause of the patient’s clumsy hand, reduced left arm movement, masked face, and lethargy. Similar to what was seen in previous reports,7,13 the patient’s parkinsonism dramatically improved after successful embolization. This made our diagnosis of DAVF-induced parkinsonism even more certain. Interestingly, progressive cognitive dysfunction or dementia, except for parkinsonism, was frequently mentioned in previous reported DAFV cases. Although Lee and colleagues reported an isolated parkinsonism-related DAVF case of a 60-year-old woman without clinically noticeable cognitive dysfunction; the patient’s mini-mental status examination score was only 15.9 In contrast, our patient showed perfect performance in his mini-mental status examination. This might be because his frontal lobes were not involved in this pathologic venous abnormality, as shown in Figure 1. For DAVF patients presenting with parkinsonism, bruit and pulsating tinnitus is an important clue.
When considering Parkinson disease and parkinsonism, the clinicians should always keep DAVF in mind for differential diagnosis. Early diagnosis of DAVF not only reverses the clinical course of parkinsonism but also decreases the risk of possible intracranial hemorrhage.
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Keywords:Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.
angiography; brain; etiology