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Brain (Cerebral) Involvement in Scleroderma

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
Systemic Scleroderma
—Vascular Headache
—What is Vertigo?
Localized Scleroderma
Personal Stories
Related Links

Overview of Brain Involvement in Scleroderma

There are two main types of scleroderma: localized (such as morphea and linear) and systemic (such as CREST, limited, diffuse, and overlap.) (Also see: What is Scleroderma?, Types of Scleroderma and Symptoms of Scleroderma)
In a small study of 30 Russian patients presented at EULAR 2004, the systemic forms of scleroderma were associated with brain (cerebral) problems, such as chronic insufficiency of brain circulation. This research is preliminary and would need to be validated by larger studies before symptoms such as cognitive impairment, migraines and vertigo would be included in the diagnosis, evaluation, or classification of systemic scleroderma. See Systemic Scleroderma Brain Involvement.
Localized scleroderma, particularly linear scleroderma, has recently been associated with both focal and progressive brain lesions, progressive brain atrophy, epilepsy, neurological symptoms, intracerebral inflammatory processes, migraine headaches, and trigeminal neuralgia. Therefore, neuroimaging studies should be considered in all patients with linear scleroderma, and certainly when neurological symptoms occur. See Localized Scleroderma Brain Involvement.

Systemic Scleroderma Brain Involvement

In a small study of 30 Russian patients presented at EULAR 2004, the systemic forms of scleroderma were associated with brain (cerebral) problems, such as chronic insufficiency of brain circulation. This research is preliminary and would need to be validated by larger studies before symptoms such as cognitive impairment, migraines and vertigo would be included in the diagnosis, evaluation, or classification of systemic scleroderma.
Cerebral sinus thrombosis in scleroderma: a case report. We discuss a scleroderma patient with high titter of anticardiolipin antibody who developed to cerebral sinus thrombosis and cerebellum infarction. PubMed. Acta Med Iran. 2012;50(4):288-91.
Neuropsychiatric manifestations in systemic sclerosis. Until recently, systemic sclerosis (SSc) was thought to spare the central nervous system (CNS). If morphological CNS abnormalities could be linked to neuropsychiatric manifestations, it would be possible to identify neuropsychiatric scleroderma as we can now identify neuropsychiatric systemic lupus erythematosus. D Launay (PubMed) Presse Med. 2010 May;39(5):539-47.
Brain magnetic resonance imaging findings in patients with systemic sclerosis. Asymptomatic as well as symptomatic central nervous system ischemic vasculopathy is not uncommon in systemic sclerosis patients and MRI is considered a sensitive noninvasive screening tool for early detection of CNS involvement in patients with systemic sclerosis. Mohamed RH. (PubMed) Int J Rheum Dis. 2010 Feb 1;13(1):61-7.
Cognitive and cerebral hemodynamic impairment in scleroderma patients. These findings show the presence of reduced performances of executive functions in scleroderma patients. The associated alteration of cerebrovascular reactivity (CVR) in the absence of other apparent causes of cerebrovascular impairment suggests that cognitive problems may be related to an alteration in cerebral perfusion regulation specifically linked to the disease. European Journal of Neurology, Volume 16, Number 12, December 2009.
Cerebral Vasculopathy Is Associated with Severe Vascular Manifestations in Systemic Sclerosis. Severe cerebral vasculopathy is associated with severe vascular manifestations in SSc patients. SSc patients with severe vascular complications should undergo neuroradiological imaging assessment of brain involvement. Benjamin Terrier. The Journal of Rheumatology. vol. 36 no. 7 1486-1494. July 2009. (Also see: Vascular Involvement)
Cognitive and cerebral hemodynamic impairment in scleroderma patients. These findings show the presence of reduced performances of executive functions in scleroderma patients. Giuliodori G. (PubMed) Eur J Neurol. 2009 Dec;16(12):1285-90.
Systemic sclerosis and superficial siderosis of the central nervous system: casuality or causality? Neurological complications, both central and peripheral, are thought to be rare and coincidental in systemic sclerosis; however over the past decade, some authors have reported a high incidence (up to 40%) of neurological involvement in the disease. S. Simeoni. (PubMed) Rheumatol Int. 2008 (Also see: Neurological Involvement)
Vestibular Balance Disorders: Vertigo, Motion Sickness, and Dizziness MedicineNet.

What is a Vascular Headache?

Vascular Disease and Systemic Scleroderma. The vascular system is our circulatory system. It consists of all the vessels that carry blood and lymph through the body, to and from organs. Vessels include arteries, veins, and capillaries. Any condition that affects the vascular system, all of in part, is considered a vascular disease. ISN.
Overview
Vascular in Scleroderma
Causes
Types in Scleroderma
In Other CTDs
Biomarkers
Angiogenesis
Hypoxia
VEGF
Personal Stories
Related Information
Vascular Headaches Vascular headaches are usually throbbing in character, and physical exertion increases the pain. Included under the classification of vascular headaches are migraine headaches, cluster headaches, and toxic headaches. All involve dilation, or a swelling of the blood vessels. National Headache Foundation.

What is Vertigo?

What is Vertigo? Vertigo is a symptom, not a disease. The term vertigo refers to the sensation of spinning or whirling that occurs as a result of a disturbance in balance (equilibrium). It also may be used to describe feelings of dizziness, lightheadedness, faintness, and unsteadiness. The sensation of movement is called subjective vertigo and the perception of movement in surrounding objects is called objective vertigo. Neurology Channel.
Goggles-and-laptop device might help detect some strokes. Researchers believe that someday, doctors may be able to use specially-equipped laptops and smartphones to figure out if sudden-onset dizziness in patients is the result of a stroke, or of a (more likely) benign disturbance in the inner ear. Chicago Tribune 03/05/2013.

Overview of Localized Scleroderma Brain Involvement

Localized scleroderma has also recently been associated with brain involvement, particularly with linear scleroderma and the type known as scleroderma en coup de sabre or Parry Romberg's syndrome. Findings include focal and progressive brain lesions, progressive brain atrophy, epilepsy, neurological symptoms, intracerebral inflammatory processes, migraine headaches, and trigeminal neuralgia. Therefore, neuroimaging studies should be considered in all patients with linear scleroderma, and certainly when neurological symptoms occur.
Severe epilepsy preceding by four months the onset of scleroderma en coup de sabre. We describe the case of a 6-year-old boy who presented partial complex seizures, with status epilepticus, four months before the appearance of sclerodermatous skin lesions on the face. Sartori S. (PubMed) Clin Exp Rheumatol. 2009 May-Jun;27(3 Suppl 54):64-7.
Localized Scleroderma: Linear/En Coup de Sabre Linear Scleroderma can appear as a long streak resembling a deep sabre wound — often called "en coup de sabre." ISN.
Also see: Linear Scleroderma, En Coup de Sabre and Parry Romberg's Syndrome ISN.

Media and Personal Stories: Cognitive, Headaches and Vestibular Disorders

Migraines show a link to gluten in some cases. Migraines can be a sign of celiac disease, and celiac patients had a greater incidence of headaches — and severe headaches, at that — than a control group. Los Angeles Times, 04/25/2014.
New promise for migraine sufferers. Now that scientists know that migraine is a neurological disorder rather than a vascular one, they are on the hunt for medicines that don't affect the cardiovascular system. Los Angeles Tmes, 03/28/2014.
Personal Stories. There are many possible causes of cognitive problems, brain fog, headaches, migraines, dizziness, balance problems and vertigo. Just because these stories are listed here does not mean their particular symptoms are caused by scleroderma or by vascular complications of scleroderma or other illnesses. ISN.

Related Links

Antiphospholipid Syndrome ISN.
Autoimmune Ear Disease ISN.
Cardiac Involvement ISN.
Chronic Fatigue Syndrome/Fibromyalgia ISN.
Gastrointestinal Involvement Gastrointestinal symptoms occur in a variety of neuromuscular diseases that may affect the neural access from the brain down to the peripheral nerve. ISN.
Scleroderma and Hypertension ISN.
Multiple Sclerosis ISN.
Neurological Involvement in Systemic Sclerosis ISN.
Raynaud's Raynaud's is an extreme spasm of blood vessels in response to cold or stress. The fingers and/or toes become white and/or blue, and may become red on re-warming. ISN.
Vasculitis is a general term for a group of diseases that involve inflammation in blood vessels. ISN.
 
Go to Brain Involvement: Stories
 

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