So where to start with this one... well I have had headaches for as long as I can remember.
Some have been mild and some have just been migraines that stop me for days... It felt like someone stabbing me with a knife or needle in the back or front part of my head constantly and enver stopping.. i could find no relief at all it seemed.
As most people do I went to doctor after doctor and all they wanted to do was give me medication... one doctor did tell me he thought I had cluster migraines... that was nice to know. At least it was something more than here take some pain medicine.
Then it happened...I guess I remember it started sometime in 2006 I noticed things changing. I couldn't remember things are well as I use to, my balance was getting to be off a bit, my headaches were more frequent... but I didn't really think much of it. I chalked it up to age, stress, work and family stuff.
By November 2007 I finally had enough and went to see my doctor after having fainted several times at work and experiencing several reoccurring headaches that were very odd.
I would start getting nauseated and have this feeling in the base of my skull like someone was fanning a feather back and forth in it. Then this harsh shooting pain would rush forward and I would be hit with the worst migraine I can imagine. I would get light headed, and blurred vision and sometimes lose balance... I passed out a few times... there were even times I noticed my left arm would tingle and be numb. I also began to have problems with high blood pressure... I was put on bp meds.... my doctor sent me for a ct scan and nothing came back. This confused him so he ordered a MRI be done and two days later I get a call at work.
My doctor tells me I have to get to a neuro specialist immediately. Well this terrified the crap out of me... he said he wasn’t sure but thought it was a vascular problem in my brain but he wanted me to see a specialist.
I wasn't impressed with the specialist but he did order an MRA done which proved something odd... it seemed that the right main artery in my brain was totally blocked... I had no blood flow at all on that side. Um hearing this I was terrified... he immediately put me on coumadine and told me to be very careful and to have someone with me at all times and have an emergency plan to get here ASAP if something happened. Um thanks this scared me even more....
After seeing him for 3 months and getting no results from him other than a weak bank account I finally found an amazing neurologist at Kirkland clinic in Birmingham, Al Dr. John Brockington.
This man was my medical dream come true. He took time to talk with me and figure this out with me and not just scare me... he came to the conclusion that my nausea and tingling and numbness was more than likely mini strokes or seizures and put me on seizure medication.... he also took me of coumadine and on aspirin daily.... within 6 months he did another MRA and ironically it showed I somehow had blood flow on the right side but the vessels were not going through the main artery. They were infact rerouting themselves to get where they needed to get blood to without it.... we were shocked but thrilled it was a positive note...
He kept me on the bp meds, seizure meds and aspirin and I have been without problems for almost 3 years now. Until now...
I gave birth to my son in October 2010 and ever since his birth I have been having issues again with headaches, dizziness, a blurred vision as well as forgetting stuff and confusion of sorts...
So back to the neurologist I go. I am having to use a different doctor because I am not as close to Birmingham anymore but hopefully we can figure this out.... it seems from what I am told so far I have blockage again but we are waiting for the rest of the test results and to decide what to do....
What concerns me is not much is known about this medical issue and there isn't much out there to read about it...
I have researched and researched and can not find very much at all and it doesn’t seem too many neurologist know much about it either.
So I wanted to do this blog to give other an idea of what they might have to deal with if they have this condition...
Symptoms I have had: constant headaches on one side in front. They start at back on skull and move forward very fast and then centralize to one side of front area.... very hard to get rid of or any relief from with Tylenol or Excedrin... will be followed with nausea, dizziness and possible fainting, blurred vision, tingling or numbness in arm or legs, easily confused, forgetful of things, easily frustrated...
But if you do have any further information about this condition please email me as I would like to know more about it if there is anything or any advancements...
Yes it has been awhile... am still having the issue with headaches, dizzy, nausea at times but lots more numbness and now pain in my left side leg and arm, am also still fogetting stuff alot and getting frustrated easily and confused... it is so frustrating because i know i was not like this several years ago so what caused this to trigger and start up? What got this going??? Why has it affected me now and Goddess forbid will it affect my children as it does me? I most certainly hope not.... I try my best not to show my fears and worries of this and try to hide it as best as I can and it is easy to do for the most part but when I get alone I just let it all out.... there are nights I cry myself to sleep worrying that I might not wake the next day to see my children or feel their hugs or see their smiles, hear their laughter... see my husbands face and feel his hand as it grasps mine.... it is just terrifying and i know he worries too but i try to be so strong....
It is now March 2012 and I have been to see my neuro in Dothan again. We did another MRI/MRA and EEG and I got the results today (3-5-2012).... I have 3 artieries on my right side blocked (the carotid, the middle cerebral and the anterial cerebral) it seems some changes have occurred... the main artery on the right side is still blocked as it has been since 2008 that we know of and now the front main artery on the right side is block as well. Also the main artery in the neck leading to the brain is blocked as well but luckily there are no signs of aneurisms or forming of aneurisms.... the EEG did show increase signs of strokes and so my neuro has made the decision to send me to the Mayo Clinic in Jacksonville, FL for more testing and to see an expert I guess. I really don't know what to think or do at this point but put it in their hands and know that the Lady knows what she is doing and will be with me giving me strength through this. SO MOTE IT BE!!!!
so after going back to see Dr. Ghori he sugggested that I go to the Mayo clinic as he is at a loss for what to do and what this truely is. He does NOT feel it is Neurovasculitis but something more serious like MoyaMoya... so he will make me an appointment... a few days later i hear from his office that Mayo clinic will contact me for appointment and within an hour they call me.. that was fast.... i have an appointment for April 30th.. this sucks because my daughters 13th bday is the 29th but I'll figure it out. Jacksonville Mayo Clinic is the closest so off to Florida I go..
We headed to Jacksonville on the 29th of April, 2012 and spent the night. I was so nervous and scared I didn't know if I was going to keep it together. my appointment was at noon. it was discussed that since we were so close to the atlantic we needed to visit it before heading back home....so we go to the Mayo Clinic and I meet with Dr. Eidelman and he was awesome. I just love him. He deffinately is old school and knows his stuff.. he has me do alot of stand up tests, memory tests and such and we looked at my MRI/MRA i brought from the SAMC in Dothan and he said "it didn't look good"... that scared me to death and i started to cry. who wouldn't.... he told me it looked like MoyaMoya to him and he explained this disease to me and how it was discovered.... i had done some research on it because Dr. Ghori had mentioned last year he thought i might be... (Good call Dr. Ghori)..... so Dr. Eidelman said he wanted me to stay another night and come tomorrow for blood work and a CT Perfussion and wanted to get an angiogram also before going further... he also wanted me to meet with his team of neuro surgeons to get their opinions.. well i mean more than one opinion is better to me so yep we stayed another night....i met with Dr. Hanel who is a neursurgeon in Eidelman's office and he said the same thing.. it looked to be MoyaMoya....i held it back but wanted to cry....all i could think of were my husband and my kids..... what about them? this isn't fair to them.... Goddess what will happen?! so then Dr. Hanel shows me the Mri/Mra and what he sees and doesnt and what needs to happen and we schedule a CT Perfusion and Angiogram.... my Ct Perfusion is in a week and the angiogram is in 2 weeks and then if all shows what they suspect then surgery will happen.. a cerebral biopsy...
terrifying.. YES...so for now I sit and wait for the CT Perfusion to be done and hope I am ok till then and until they figure this out and fix me... i have faith and trust in these doctors and know the Goddess will see them strong and knowledgable enough to help me...I still have constant headaches and am dizzy and nauseated....... Dr. Hanel also said the Mri/Mra showed some damage to certain areas of my brain due to a stroke but we can't detect when it happened.. it is small and minor areas this happened to but we are trying to keep it from being a big thing and a major one....
MoyaMoya is such a rare disease and so little is known about it that It worries me to no end.,......
Schematic representation of the circle of Willis, arteries of the brain, and brain stem.
so after going back to see Dr. Ghori he sugggested that I go to the Mayo clinic as he is at a loss for what to do and what this truely is. He does NOT feel it is Neurovasculitis but something more serious like MoyaMoya... so he will make me an appointment... a few days later i hear from his office that Mayo clinic will contact me for appointment and within an hour they call me.. that was fast.... i have an appointment for April 30th.. this sucks because my daughters 13th bday is the 29th but I'll figure it out. Jacksonville Mayo Clinic is the closest so off to Florida I go..
We headed to Jacksonville on the 29th of April, 2012 and spent the night. I was so nervous and scared I didn't know if I was going to keep it together. my appointment was at noon. it was discussed that since we were so close to the atlantic we needed to visit it before heading back home....so we go to the Mayo Clinic and I meet with Dr. Eidelman and he was awesome. I just love him. He deffinately is old school and knows his stuff.. he has me do alot of stand up tests, memory tests and such and we looked at my MRI/MRA i brought from the SAMC in Dothan and he said "it didn't look good"... that scared me to death and i started to cry. who wouldn't.... he told me it looked like MoyaMoya to him and he explained this disease to me and how it was discovered.... i had done some research on it because Dr. Ghori had mentioned last year he thought i might be... (Good call Dr. Ghori)..... so Dr. Eidelman said he wanted me to stay another night and come tomorrow for blood work and a CT Perfussion and wanted to get an angiogram also before going further... he also wanted me to meet with his team of neuro surgeons to get their opinions.. well i mean more than one opinion is better to me so yep we stayed another night....i met with Dr. Hanel who is a neursurgeon in Eidelman's office and he said the same thing.. it looked to be MoyaMoya....i held it back but wanted to cry....all i could think of were my husband and my kids..... what about them? this isn't fair to them.... Goddess what will happen?! so then Dr. Hanel shows me the Mri/Mra and what he sees and doesnt and what needs to happen and we schedule a CT Perfusion and Angiogram.... my Ct Perfusion is in a week and the angiogram is in 2 weeks and then if all shows what they suspect then surgery will happen.. a cerebral biopsy...
terrifying.. YES...so for now I sit and wait for the CT Perfusion to be done and hope I am ok till then and until they figure this out and fix me... i have faith and trust in these doctors and know the Goddess will see them strong and knowledgable enough to help me...I still have constant headaches and am dizzy and nauseated....... Dr. Hanel also said the Mri/Mra showed some damage to certain areas of my brain due to a stroke but we can't detect when it happened.. it is small and minor areas this happened to but we are trying to keep it from being a big thing and a major one....
MoyaMoya is such a rare disease and so little is known about it that It worries me to no end.,......
Moyamoya disease is a progressive, occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis and the arteries that feed it.[1] The image below is a schematic representation of the circle of Willis, the arteries of the brain, and the brainstem. (See Etiology.)
Schematic representation of the circle of Willis, arteries of the brain, and brain stem.
The term moyamoya (Japanese for "puff of smoke") refers to the appearance on angiography of abnormal vascular collateral networks that develop adjacent to the stenotic vessels. The steno-occlusive areas are usually bilateral, but unilateral involvement does not exclude the diagnosis. (See Workup.)
Pathologically, moyamoya disease is characterized by intimal thickening in the walls of the terminal portions of the internal carotid vessels bilaterally. The proliferating intima may contain lipid deposits. The anterior, middle, and posterior cerebral arteries that emanate from the circle of Willis may show varying degrees of stenosis or occlusion. This is associated with fibrocellular thickening of the intima, waving of the internal elastic lamina, and thinning of the media. (See Etiology, Workup, Treatment, and Medication.)
Numerous small vascular channels can be seen around the circle of Willis. These are perforators and anastomotic branches. The pia mater may also have reticular conglomerates of small vessels.
The cause of moyamoya disease is not known. The disease is believed to be hereditary. Fukui reported a family history in 10% of patients with the disorder. Moreover, Mineharu suggested that familial moyamoya disease is autosomal dominant with incomplete penetrance that depends on age and genomic imprinting factors.[2] Genetically, susceptibility loci have been found on 3p, 6p, 17q, and band 8q23. Mineharu et al have found a specific gene locus, q25.3, on chromosome 17.[3]
People with moyamoya disease have been found to have a higher incidence of elevated thyroid antibodies.[4] While this is an association in some individuals, the significance is not clear. However, it suggests that immune abnormalities may play some role in moyamoya disease.
Associated diseases
Although moyamoya disease may occur by itself in a previously healthy individual, many disease states have been reported in association with moyamoya disease, including the following:
- Infections - Leptospirosis and tuberculosis
- Hematologic disorders - Aplastic anemia, Fanconi anemia, sickle cell anemia, and lupus anticoagulant
- Congenital syndromes - Apert syndrome, Down syndrome, Marfan syndrome, tuberous sclerosis, Turner syndrome, von Recklinghausen disease, and Hirschsprung disease
- Vascular diseases - Atherosclerotic disease, coarctation of the aorta and fibromuscular dysplasia, cranial trauma, radiation injury, parasellar tumors, and hypertension
These associated conditions may not be causative, but they do warrant consideration due to their impact on treatment.
A study indicated that the prevalence of moyamoya disease in California and Washington was 0.086 case per 100,000 population.[6] In this study, the breakdown based on ethnicity as ratio to whites was 4.6 for Asian Americans, 2.2 for African Americans, and 0.5 for Hispanics.
The incidence of moyamoya disease is highest in Japan. The prevalence and incidence of the disorder there has been reported to be 3.16 cases and 0.35 case per 100,000 people, respectively.
Race-, sex-, and age-related demographics
Moyamoya disease occurs primarily in Asians but can also occur (with varying degrees of severity) in whites, blacks, Haitians, and Hispanics.
The female-to-male ratio of moyamoya disease is 1.8:1. Ages for patients with moyamoya disease range from 6 months to 67 years, with the highest peak in the first decade and smaller peaks in the third and fourth decades.
Death from with moyamoya disease is usually from hemorrhage. The outcome of the disease depends on the severity and nature of the hemorrhage; the prognosis depends on recurrent attacks.
Mortality rates from moyamoya disease are approximately 10% in adults and 4.3% in children. About 50-60% of affected individuals experience a gradual deterioration of cognitive function, presumably from recurrent strokes.
Patients with moyamoya disease who present for treatment while symptoms are evolving have a better prognosis than do those who present with static symptoms (which probably indicate a completed stroke).
Children and adults with moyamoya disease may have different clinical presentations. The symptoms and clinical course vary widely, with the disease ranging from being asymptomatic to manifesting as transient events to causing severe neurologic deficits. Adults experience hemorrhage more commonly; cerebral ischemic events are more common in children.
Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures. Mental retardation or persistent neurologic deficits may be present.
Adults may have symptoms and signs similar to those in children, but intraventricular, subarachnoid, or intracerebral hemorrhage of sudden onset is more common in adults.
Physical examination
Physical examination findings depend on the location and severity of the hemorrhage or ischemic insult.
Misdiagnosis and delayed diagnosis of moyamoya disease are particular pitfalls in the treatment of patients with this disorder. Misdiagnosis can occur easily if the physician does not incorporate moyamoya disease into the differential diagnosis of any patient presenting with stroke. How high moyamoya disease is ranked in the differential depends on presence of atypical features such as young age and absence of obvious risk factors for stroke.
If moyamoya disease is not considered seriously, then appropriate diagnostic tests may not be performed and a delay in diagnosis could result. Because definitive treatment may be surgery, any delay could allow unnecessary progression of disease.
If an ischemic stroke that is being treated with antiplatelet agents or anticoagulants does not respond to therapy, then moyamoya disease should be considered as a possible etiology. This is especially true if results of a hypercoagulability profile are unremarkable.
Physicians practicing in the community who encounter atypical stroke presentations should not hesitate to seek consultation with a stroke specialist or even to transfer a patient to a facility equipped to care for complex cases.
Several studies may be indicated in patients with moyamoya disease. In a patient with stroke of unclear etiology, a hypercoagulability profile may be helpful. Significant abnormality in any of the following is a risk factor for ischemic stroke:
- Protein C
- Protein S
- Antithrombin III
- Homocysteine
- Factor V Leiden
The erythrocyte sedimentation rate (ESR) can be obtained as part of the initial workup of possible vasculitis. However, a normal ESR does not rule out vasculitis.
Thyroid function and thyroid autoantibody levels have been shown to be elevated in a significant percentage of pediatric patients with moyamoya disease.[7] Therefore, monitoring these studies is indicated.
Cerebral angiography is the criterion standard for the diagnosis of moyamoya disease. The following findings support the diagnosis:
- Stenosis or occlusion at the terminal portion of the internal carotid artery or the proximal portion of the anterior or middle cerebral arteries
- Abnormal vascular networks in the vicinity of the occlusive or stenotic areas
- Bilaterality of the described findings (although some patients may present with unilateral involvement and then progress)
Magnetic resonance angiography (MRA) can be performed. Any of the above findings on MRA may preclude the need for conventional angiography.
Pharmacologic therapy for moyamoya disease is disappointing. Therapy is primarily directed at complications of the disease. If intracerebral hemorrhage has occurred, then management of hypertension (if present) is imperative. In cases of severe stroke, intensive care unit (ICU) monitoring is indicated until the patient's condition stabilizes. If the patient has had an ischemic stroke, consider anticoagulation or antiplatelet agents.
Activity
Rehabilitation with physical therapy, occupational therapy, and speech therapy should be considered, depending on the neurologic impairment. The extent of therapy can range from bedside treatment to full, comprehensive inpatient rehabilitation. The latter would include physical, occupational, speech, and cognitive therapy. The condition of the patient, including active comorbidities, dictates his or her involvement in rehabilitation therapy.
The rationale behind the administration of anticoagulation and antiplatelet agents is the prevention of further strokes, especially in stenotic vessels (where further infarction can occur if occlusion progresses).
These medications are not approved by the US Food and Drug Administration (FDA) specifically for use in moyamoya disease. Therefore, the decision to treat patients with anticoagulants such as heparin (and, in some cases, warfarin, for long-term anticoagulation) or antiplatelet agents such as aspirin rests on the following: angiogram findings, severity of stroke, and risk/benefit analysis by physicians who are experienced in stroke treatment.
As previously stated, patients with moyamoya disease who present for treatment while symptoms are evolving have a better prognosis than do those who present with static symptoms (which probably indicate a completed stroke).
Various surgical procedures have been used in the treatment of moyamoya disease, including the following:
- Superficial temporal artery–middle cerebral artery (STA-MCA) anastomosis
- Encephaloduroarteriosynangiosis (EDAS)[8]
- Encephaloduroarteriomyosynangiosis (EDAMS)
- Pial synangiosis
- Omental transplantation
These procedures can be divided into 2 groups depending on whether they involve direct or indirect anastomosis. Which of these procedures is most effective remains controversial. Sufficient evidence suggests that surgical revascularization procedures result in some symptomatic benefits along with demonstration of improved blood flow. Direct and/or combined procedures provide improved vascularization. However, data proving sustained or improved long-term outcomes are insufficient.[9, 10]
STA-MCA anastomosis is very difficult in children younger than 2 years because of the small diameter of the STA. In these cases, EDAS may be more suitable. This procedure sometimes has failed because of poor revascularization. Hoffman suggested that this is due to the presence of atrophy and a layer of spinal fluid between the pia and the arachnoid tissue.[11] Division of the arachnoid membrane and placement of the STA directly on the pial membrane help to avoid the problem. In cases of EDAS failure, EDAMS can be considered.
Drug therapy for moyamoya disease depends on the particular manifestations of the disease. For hemorrhage, therapy revolves around the management of hypertension (if present).
For ischemic stroke, anticoagulation with heparin or warfarin may be considered. Safety and efficacy have not been fully established for these drugs, and careful analysis of risk and benefits is needed. These drugs could be useful if thrombosis of vessels is present, but they do not alter the natural history of the disease and they considerably increase the risk of hemorrhage with large strokes.
The same considerations are true for aspirin and other antiplatelet agents. Treatment with anticoagulation or antiplatelet agents should be pursued only after consultation with a neurologist who is experienced in stroke management.
These agents are given for the prevention of further thrombosis and potential infarction of the brain. Caution: Anticoagulation is of unproven benefit in ischemic stroke associated with moyamoya disease. This therapy is therefore considered to be empirical.
Heparin
Heparin is administered intravenously; it is frequently given with initial bolus in cardiac situations. In stroke, bolus not recommended. The target dose is aimed at maintaining an activated partial thromboplastin time (aPTT) of 1.5-2 times control. A computed tomography (CT) scan of the brain must be done prior to any anticoagulant use to rule out preexisting intracranial hemorrhage.
Warfarin (Coumadin, Jantoven)
Warfarin, which is administered orally, is used if long-term anticoagulation is needed. The international normalized ratio (INR) is followed, with a target range of 2-3. A CT scan of the brain must be done prior to any anticoagulant use to rule out preexisting intracranial hemorrhage.
These agents can be considered to help prevent future ischemic strokes. As with anticoagulation, aspirin is of unproven benefit in moyamoya disease; its use is considered empirical.
Aspirin (Ecotrin, Ascriptin Maximum Strength, Ascriptin, Bayer Aspirin)
Aspirin's efficacy in preventing stroke relies on the inhibitory effect of aspirin on platelet function. This presumably helps to prevent thrombus formation and propagation.
so it has been a month or more since i have first seen Dr. Eidelman and Hanel and they have done a CT Perfussion scan which hurt like hell and make me sick as crap and then a week later i had an angiogram done.. both of which confirmed I DO have moyamoya.... so it is crucial I have surgery they say... this will prevent me from having a major stroke or worse and they are actually surprised i haven't yet...
so we went ahead and scheduled surgery for the month of June, 2012 and I am terrified to death.... yeah i know everyone is scared of surgery but after researching about this and knowing what they will do and what is involved and what i will have to go through it just scares me to no end.... on the flip side Dr. Hanel has had 0% failure with this operation and is one of the to surgeons in the US for moyamoya surgery... so i feel i am in good hands and confident but still scared as hell.. who wouldn't be right!?!
I just would like to know what to expect after.. like what will happen... will i have a personality change or what will recovery really be like.. stuff like that and i know it is different for everyone but i just wish i could get answers to these things.....
i have found some blogs and places on here i can go to talk with people who have the same thing i do and hopefully i can find out stuff from them....
