Hyperhomocysteinemia and left internal jugular vein thrombosis with Meniere's symptom complex.
The author describes the case of a 68-year-old woman who experienced severe symptoms of Meniere's syndrome. Ultrasonography of the neck detected a clot in the left internal jugular vein internal jugular vein
A vein that is a continuation of the sigmoid sinus of the dura mater and unites behind the cartilage of the first rib with the subclavian vein to form the brachiocephalic vein. . The patient was hospitalized and anticoagulated, and her symptoms soon resolved. The author speculates that the clot-induced hemodynamic he·mo·dy·nam·ics
n. (used with a sing. verb)
The study of the forces involved in the circulation of blood.
he changes led to venous insufficiency in the vein of the paravestibular canaliculus canaliculus /can·a·lic·u·lus/ (kan?ah-lik´u-lus) pl. canali´culi [L.] an extremely narrow tubular passage or channel.canalic´ular
apical canaliculus and ultimately caused the patient's symptoms. The blood work-up revealed that the patient had an elevated fasting homocysteine level, which is a known risk factor for thrombus formation.
Hyperhomocysteinemia is a risk factor for both arterial and venous thrombosis. In this article, the author describes the case of a patient who developed incapacitating Meniere's symptom complex. The work-up revealed a left internal jugular vein thrombosis, and it was later found that the patient had hyperhomocysteinemia. The author postulates that the hyperhomocysteinemia predisposed this patient to clot formation in the left internal jugular vein and this in turn led to the development of the Meniere's symptoms. Once the patient was anticoagulated, her symptoms resolved.
A 68-year-old white woman had been initially treated by the author for vertigo, left-sided tinnitus, and left-sided aural fullness. During her episodes of vertigo, she experienced nausea, vomiting, and decreased hearing in her left ear. She had no history of trauma or instrumentation to her head and neck. After conservative management with meclizine meclizine /mec·li·zine/ (mek´li-zen) an antihistamine used as the hydrochloride salt as an antinauseant in motion sickness and to manage vertigo associated with disease affecting the vestibular system. and methylprednisolone methylprednisolone /meth·yl·pred·nis·o·lone/ (-pred-nis´ah-lon) a synthetic glucocorticoid derived from progesterone, used in replacement therapy for adrenocortical insufficiency and as an antiinflammatory and immunosuppressant; also failed to alleviate her symptoms, she was referred to an otolaryngologist.
During the initial referral consultation, findings on a complete head and neck evaluation were within normal limits. Audiometry demonstrated a low-frequency sensorineural hearing loss Sensorineural hearing loss
Hearing loss caused by damage to the nerves or parts of the inner ear governing the sense of hearing.
Mentioned in: Tinnitus
sensorineural hearing loss on the left and some reduction in the patient's speech discrimination scores (84% on the left; 100% on the right). She was diagnosed with Meniere's symptom complex and given dietary instructions.
Despite these measures, the Meniere's symptoms incapacitated the patient to the point where she was unable to work. She next underwent magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures. (MRI) of the brain and magnetic resonance angiography Magnetic resonance angiography
A noninvasive diagnostic technique that uses radio waves to map the internal anatomy of the blood vessels.
Mentioned in: Cerebral Aneurysm
magnetic resonance angiography (MRA) of the neck. Findings on MRI were essentially unremarkable, but MRA revealed a possible 60 to 70% stenosis of the right internal carotid artery. The patient was then sent for a Doppler ultrasound study of the carotids. Ultrasonography did not detect any hemodynamic stenosis, but it did show a thrombus in the left proximal internal jugular vein (figure 1). Her protein C and S levels were within normal limits. The patient was hospitalized and anticoagulated with 1 mg/kg of enoxaparin subcutaneously every 12 hours. She was then started on warfarin to achieve an international normalized ratio International Normalized Ratio Hematology A method of reporting prothrombin time–PT results for Pts receiving oral anticoagulant therapy; the INR is defined by the formula, PTPatient/PTMNPT between 2.0 and 2.5. Once the patient was anticoagulated, her Meniere's symptoms resolved.
[FIGURE 1 OMITTED]
During warfarin anticoagulation, a hematologic hematological, hematologic
pertaining to or emanating from blood cells.
total and differential white cell counts, hematocrit estimation, erythrocyte count. evaluation yielded normal values for antithrombin III, lupus anticoagulants, prothrombin prothrombin
Carbohydrate-protein compound in plasma essential to coagulation. In response to bleeding, a complex series of clotting-factor interactions leads to its conversion by thromboplastin to thrombin, which transforms fibrinogen in plasma into fibrin. gene mutations, antinuclear antibodies, and anticardiolipin antibodies. However, her fasting homocysteine level was elevated at 20.3 [micro]mol/L (range of normal: 4.5 to 12.4). She was started on 1 mg/ day of folic acid and a once-daily multivitamin. She was maintained on warfarin for 6 months and then switched to aspirin at 325 mg/day.
A follow-up ultrasound approximately 2 months after the start of anticoagulation showed no clot in the left internal jugular vein (figure 2). An ultrasound performed approximately 3 months after warfarin was discontinued again did not demonstrate a left internal jugular vein clot (figure 3). The patient remained free of her Meniere's symptoms during her 6 months on anticoagulation, and she remained symptom-free on a regimen of 1 aspirin per day at 15 months of follow-up.
[FIGURES 2-3 OMITTED]
Hyperhomocysteinemia can predispose patients to deep vein thrombosis A blood clot (thrombos) in a vein deep within the muscle, typically in the thigh or calf. It is caused by disease or the lack of activity such as sitting for hours at a computer screen. ; in this case, the patient's left internal jugular vein was affected. The author postulates that the clot in the left internal jugular vein caused an increase in venous pressure that resulted in venous insufficiency in the vestibular organs. This in turn led to endolymphatic hydrops and the subsequent development of Meniere's symptom complex.
The vein of the paravestibular canaliculus (VPVC) runs parallel to the endolymphatic duct and drains the major portion of the semicircular canals and part of the utricle utricle /utri·cle/ (u´tri-k'l)
1. any small sac.
2. the larger of the two divisions of the membranous labyrinth of the internal ear. . The mean width of the paravestibular canaliculus is 0.12 mm. (1) The VPVC receives several veins from the endolymphatic sac and drains into the inferior petrosal sinus inferior petrosal sinus
A paired sinus of the dura mater that connects the cavernous sinus with the superior bulb of the internal jugular vein. or directly into the jugular bulb (figure 4).
[FIGURE 4 OMITTED]
Some research has shown that there are certain variations in the pattern of venous drainage of the vestibular organs. Mazzoni demonstrated that in a certain percentage of human temporal bones, one or two of the vestibular veins drain through the VPVC rather than serving as tributaries of the inferior cochlear cochlear
pertaining to or emanating from the cochlea.
the coiled portion of the membranous labyrinth located inside the cochlea; contains endolymph.
see Table 14. vein. (2) Gussen suggested that in these temporal bones, venous insufficiency in the VPVC might produce endolymphatic hydrops. (3) Gussen postulated that increased venous pressure and insufficiency of the vestibular organs could represent the common endpoint of many of the entities known to cause Meniere's disease, although the exact mechanism of increased vestibular venous pressure may differ in each circumstance.
The author postulates that the clot in the left internal jugular vein in this patient altered the blood flow dynamics, which led to increased venous pressure and the resultant venous insufficiency in the VPVC. It is known that the VPVC can drain directly into the jugular bulb. In this patient, the resultant hemodynamic alteration occurring in the left jugular bulb may have disturbed the venous drainage of the endolymphatic duct and/or sac, thereby producing endolymphatic hydrops. In fact, in an article on the surgical lowering of the high jugular bulb in the treatment of 13 patients with Meniere's disease and pulsatile tinnitus, Couloigner et al postulated that the symptoms in these patients were caused by compression of venous drainage of the endolymphatic duct and/or sac by the high jugular bulb. (4) After they surgically lowered the jugular bulb, the symptoms improved, thus adding credence to the importance of venous drainage of the vestibular organs through the VPVC. In the case described here, the effect of the clot in the left internal jugular vein was similar to the effect of a high jugular bulb with respect to the way it altered blood flow dynamics.
The hematologic work-up in the patient described here revealed that she had hyperhomocysteinemia, which increases the risk of clot formation. Hyperhomocysteinemia occurs as a consequence of either (1) the inability of homocysteine to remethylate to methionine or (2) the trans-sulfuration of methionine to cysteine. Folic acid and vitamins [B.sub.6] and [B.sub.12] are important in the processes of remethylation and trans-sulfuration (figure 5). Clot formation is apt to occur in the presence of one or more of Virchow's triad (intimal intimal
pertaining to or emanating from vascular intima.
irregular mineralized masses covered by endothelium and protruding into the lumen of small arteries and arterioles of horses, especially in the intestinal injury, altered blood flow, and hypercoagulable blood). (5) Hyperhomocysteinemia can cause thrombosis either directly or indirectly. For example, Lentz wrote that homocysteine can inhibit endothelial production of nitric oxide, which has antiplatelet an·ti·plate·let
Acting against or destroying blood platelets.
directed against or destructive to blood platelets; inhibiting platelet function. activity and is known to prevent vasoconstriction vasoconstriction /vaso·con·stric·tion/ (-kon-strik´shun) decrease in the caliber of blood vessels.vasoconstric´tive
n. . (6) Lentz also reported that oxidation of homocysteine can generate reactive oxygen species reactive oxygen species,
n molecules and ions of oxygen that have an unpaired electron, thus rendering them extremely reactive. Many cellular structures are susceptible to attack by ROS contributing to cancer, heart disease, and cerebrovascular disease. , such as hydrogen peroxide, superoxide anion, and hydroxyl anion. These reactive oxygen species may impair the endothelium-dependent activation of protein C, thus predisposing a patient to thrombosis. Hyperhomocysteinemia may also stimulate a proliferation of vascular smooth muscle Vascular smooth muscle refers to the particular type of smooth muscle found within, and composing the majority of the wall of blood vessels.
Vascular smooth muscle contracts or relaxes to both change the volume of blood vessels and the local blood pressure, a mechanism that cells.
[FIGURE 5 OMITTED]
The patient described here was treated with folic acid and a daily multivitamin that contained B supplements. Although it is not known whether thrombosis can be prevented with these supplements, she remained clot-free 15 months after the initiation of therapy.
(1.) Stahle J, Wilbrand H. The paravestibular canaliculus. Can J Otolaryngol 1974;3:262-70.
(2.) Mazzoni A. Vein of the vestibular aqueduct. Ann Otol Rhinol Laryngol 1979;88(Pt 1):759-67.
(3.) Gussen R. Vascular mechanisms in Meniere's disease. Otolaryngol Head Neck Surg 1983;91:68-71.
(4.) Couloigner V, Grayeli AB, Bouccara D, et al. Surgical treatment of the high jugular bulb in patients with Meniere's disease and pulsatile tinnitus. Eur Arch Otorhinolaryngol 1999;256:224-9.
(5.) Cohen cohen
(Hebrew: “priest”) Jewish priest descended from Zadok (a descendant of Aaron), priest at the First Temple of Jerusalem. The biblical priesthood was hereditary and male. JP, Persky MS, Reede DL. Internal jugular vein thrombosis. Laryngoscope 1985;95:1478-82.
(6.) Lentz SR. Mechanisms of thrombosis in hyperhomocysteinemia. Curr Opin Hematol 1998;5:343-9.
Dr. Scaramella is a family physician in private practice in Chicago. Reprint requests: John G. Scaramella, MD, 4742 N. Austin, Chicago, IL 60630. Phone: (773) 202-8034: fax: (773) 202-8147.