| Brain (Cerebral) Involvement in Scleroderma |
| This page was written by Shelley Ensz, and has not yet been medically edited. Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean that it is due to scleroderma or that an individual patient will ever experience it. See Disclaimer. |
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| 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.) |
| 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. |
| Peripheral nervous system lesion syndromes and the mechanisms of their formation in connective tissue diseases. Systemic rheumatological diseases are often accompanied by the development of central and peripheral nervous system pathology. Lupus erythematosus and systemic scleroderma in particular are characterized by polyneuropathies and tunnel syndromes. PubMed. Neurosci Behav Physiol. 2007 Jan;37(1):1-6. (Also see: Lupus) |
| Cognitive Impairment in Systemic Sclerosis (SSc). Cognitive impairments are significantly more frequently encountered in SSc than in Rheumatoid Arthritis patients. The cause of such cognitive impairment remains to be investigated. Christine Moroni. 1873/545 ACR 2006. |
| Autoimmune brain inflammation studied. U.S. scientists say a key factor in development of brain inflammation may provide a new target for inflammatory diseases of the central nervous system. United Press International. 08/15/06. |
| Steroids and Brain Atrophy |
| Steroids and brain atrophy in multiple sclerosis. It appears that chronic low-dose treatment with corticosteroids may contribute to irreversible loss of brain tissue in a variety of autoimmune diseases. Evidence is mounting that high-dose corticosteroids may induce reversible short-term brain volume changes. Further longitudinal studies are needed to elucidate pathogenetic mechanisms contributing to brain volume fluctuations in autoimmune diseases and multiple sclerosis. PubMed. J Neurol Sci. 2005 May 5. (Also see: Multiple Sclerosis, and Medications) |
| 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. |
| Proton Magnetic Resonance Spectroscopy Reveals Central Neuroaxonal Impairment in Systemic Sclerosis. Involvement of the central nervous system (CNS) in systemic sclerosis (SSc) is rare. Evidence of neuroaxonal damage strongly suggests the existence of CNS involvement in SSc. J. Rheumatol 2006 March;33:546–51. |
| White matter hyperintensities on brain magnetic resonance in systemic sclerosis. White matter hyperintensities are more common in patients with SSc than in a CG (control group). These findings might be related to obliterative microvascular processes due to the disease. Early brain involvement in patients with SSc may occur. PubMed. Ann Rheum Dis. 2005 May;64(5):777-9. |
| Ischaemic stroke in progressive systemic sclerosis. We have documented a cerebral infarct in a PSS patient. In our opinion, the ischaemic stroke was caused by a localised autoimmune angiopathy. PubMed. Neurol Sci. 2004 Oct;25(4):230-233. |
| Hippocampal damage in mouse and human forms of systemic autoimmune disease. Taken together, these results suggest that systemic autoimmunity induces significant hippocampal damage, which may underlie affective and cognitive deficits in NP-SLE. PubMed. Hippocampus. 2004;14(5):649-61. (Also see: Lupus) |
| Cerebral vascular pathology in systemic sclerosis. The data obtained suggest that extra- and intracranial blood flow pathology in SS may, to a large extent, determine both organic brain lesion development in general and the genesis of many symptoms (headaches, vestibular disorders, etc). PubMed. Zh Nevrol Psikhiatr Im S S Korsakova. 2004;104(4):19-23. |
| Incidence of Cognitive Impairment in Systemic Sclerosis. Among the 17 patients, 13 had evidence for a cognitive impairment such as difficulties in performing two different cognitive tasks simultaneously, drawing elaboration, and memory impairment. Ten (59%) patients had a documented defect in long term memory research strategy, whereas 3 (17%) presented with short term defect. Only 3 of the 13 SSc patients who had cognitive impairment also had evidence for depression, as assessed by the MINI. C. Moroni. FRI0338 EULAR 2004. |
| Vestibular Balance Disorders: Vertigo, Motion Sickness, and Dizziness MedicineNet. |
| Magnetic Resonance Imaging (MRI) of the Brain in Patients with Systemic Sclerosis (SSC). Based on the findings of this study, albeit on a small number of patients, CNS involvement by SSc itself, does not seem to be substantiated. The need for studies in larger SSc populations is evident. A. P. Andonopoulos. AB0340 EULAR 2003. |
| What is a Vascular Headache? |
| 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. |
| Migraine Is An Independent Risk Factor For Arterial Thrombotic Events (ATE) In Systemic Lupus Erythematosus (SLE). The presence and the frequency of migraine attacks, but not the duration of migraine was associated with ATE in SLE patients. S. Appenzeller. THU0228. EULAR 2008. (Also see: Lupus) |
| Headache, Hope Through Research Not all headaches require medical attention. Some result from missed meals or occasional muscle tension and are easily remedied. But some types of headache are signals of more serious disorders, and call for prompt medical care. National Institute of Neurological Disorders and Stroke. |
| 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. |
| 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. |
| Neuroimaging findings in scleroderma en coup de sabre. Localized scleroderma en coup de sabre is associated with focal, and in some progressive, brain lesions underlying the skin atrophy. Epilepsy, when present, is related to these brain lesions. Imaging findings and histopathology indicated that the process, most likely focal inflammatory, may be progressive. PubMed. Neurology. 2004 May 11;62(9):1585-9. |
| Neuroimaging Findings in Parry Romberg Syndrome and Scleroderma En Coup Sabre. In both syndromes there were signs of cortical abnormalities underneath the skin lesions. Whether this represents a cortical dysgenesis or a progressive degenerative/inflammatory lesion remains to be determined. Follow up brain imaging multicentric collaborative studies may clarify this issue. S. Appenzeller. AB0326 EULAR 2003. |
| Linear scleroderma "en coup de sabre" coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids. Brain magnetic resonance imaging (MRI) showed focal T2 signal increases in the left frontoparietal region directly adjacent to the area of LSCS (Linear scleroderma "en coup de sabre"). Cerebrospinal fluid analysis revealed oligoclonal bands, suggesting an intracerebral inflammatory process. Subsequent pulsed corticosteroid treatment led to a remission of neurological symptoms and to a marked resolution of the MRI lesions. PubMed. J Neurol Neurosurg Psychiatry 2003 May;74(5):661-4. |
| Linear scleroderma associated with progressive brain atrophy. This study reports one case where neurological involvement may be associated with ipsilateral facial and skull involvement in disorders referred to clinically as LS 'en coup de sabre', and Parry-Romberg syndrome. Neuroimaging studies should be considered in all patients with linear scleroderma, and such studies become necessary when neurological symptoms occur. Brain Dev 2003 Jan;25(1):57-61 PubMed. |
| Linear Scleroderma en Coup de Sabre and Brain Calcification: Is There a Pathogenic Relationship? We describe a female patient with linear scleroderma en coup de sabre and a longstanding clinical history of tonic and clonic convulsions. Radiographic study showed extensive cerebral calcifications in the right occipital hemisphere, homolateral to the involved side of her face. Brain imaging studies should be routinely performed in scleroderma patients exhibiting neurological manifestations, especially seizure disorder. DIANA ELSA FLORES-ALVARADO. J Rheumatol 2003;30:193-5. |
| 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. |
| Parry-Rombergs Syndrome, aka Progressive Hemifacial Atrophy or HFA. This may be the same as Linear Scleroderma, affecting the facial area. It usually affects the left half of the face and the facial atrophy may be accompanied by other symptoms, including migraine headaches and trigeminal neuralgia. The Rombergs Connection. |
| Also see: Linear Scleroderma, En Coup de Sabre and Parry Romberg's Syndrome ISN. |
| Personal Stories: Cognitive, Headaches and Vestibular Disorders |
| 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. |
| Betty M: CREST Syndrome I was in so much pain for so long that I tried to take my own life. This only ended me up in a mental institution where I was given shock treatments that wiped out my whole memory... |
| Carla: CREST Syndrome and Fibromyalgia When I read some of these stories, they scare the devil out of me. Is this really that bad?... |
| Carol C: CREST Syndrome Scleroderma and Raynaud's have not robbed me of a fulfilling career... |
| Cedric: Linear Scleroderma, En Coup de Sabre (South Africa) I became aware of a small brown spot just above my right eye and gradually this patch became bigger and darker... |
| Cindy: Undifferentiated Connective Tissue Disease (UCTD) He seems to think that I have Systemic Lupus Erythematosus, but until my symptoms show more criteria for it, he can't call it that... |
| Debbie: CREST Here's a copy of a letter I sent to my rheumatologist.... |
| Debby: CREST Scleroderma It was then everything added up— that I did not have several different conditions, but rather they were all part of the CREST... |
| Dienne: Morphea plus possible CREST I finally saw a rheumatologist about 6 years ago, who told me I had Raynaud's and Telangiectasia (spider veins)... |
| Faith Rumph: Eosinophilia-Myalgia Syndrome (EMS) I am one of several thousand Americans who became ill from ingesting contaminated over-the-counter L-tryptophan, an amino acid dietary supplement... |
| Gail: Undifferentiated Connective Tissue Disease (UCTD) They found I had Interstitial Cystitis, a positive ANA, and gastrointestinal dysmotility... |
| Gertie: Systemic Scleroderma without Skin Involvement or Raynaud's She looked thoughtful for a moment, then quietly asked "Is there hope?" |
| Gina: Morphea or Linear Scleroderma I really try to put it out of my mind and be thankful that it is not systemic... |
| Heidi: Difficult Diagnosis of UCTD Do these symptoms resemble the beginning symptoms for others who have gone on to be diagnosed with scleroderma? |
| Jewell: Diffuse Progressive Systemic Sclerosis When I think back, I had been getting sick for a long time. I just did not know it... |
| Joan Stovall: Scleroderma Dallas, Texas I may have scleroderma, but it does not have me... |
| Jody: Fibromyalgia/Difficult Diagnosis In Sudan, I was on IV for heat stroke and food poisoning. And in Thailand, at the end of March, it all came to a crashing halt... |
| Karen M.R.: Diffuse Scleroderma and Fibromyalgia I was so glad she was there, because I sort of went into shock when the doctor said she was going to start me on chemo treatments... |
| Kathi: Scleroderma/Lupus I am forty-three years old and have not been feeling well for ten years... |
| Kellie: Overlap Scleroderma and Mixed Connective Tissue Disease (MCTD) I am so relieved to read about so many people who have had the same experiences... |
| Linda F: CREST Scleroderma, Rheumatoid Arthritis, Fibromyalgia, Hashimoto's Thyroiditis, Hematoma, and Osteomyelitis I need to tell you all the gory details so you can truly understand what a miracle it is that I am alive today... |
| Lisa P: Undifferentiated Connective Tissue Disease (UCTD) I have an appointment with a new rheumatologist on May tenth. Hopefully I will get some answers... |
| Marie: CREST About 8 years ago, I noticed my fingers were going numb, turning white, while grocery shopping... |
| Mary S: Diffuse Scleroderma When he called the internist I was in the office and I heard him say, "I think I am sending you a patient who has scleroderma but I am not sure. She has a lot of the symptoms." |
| Monica: MCTD This diagnosis is a bittersweet one for us. Yes, after seven years of hell, we now have a name... |
| Robin: Difficult Diagnosis My rheumatologist said I have scleroderma, but just lately she said now she is not sure if it is that or fibromyalgia... |
| Patty S.:Localized Scleroderma, Pulmonary Hypertension and Sjögren's I have quit my job and reduced my stress a hundred percent. I am doting on my children and my husband... |
| Shar: Lupus, Scleroderma, Sjögren's, and Fibromyalgia Was my battle with Hepatitis C worth it? Yes! |
| Sherrill: Diffuse Systemic Scleroderma (New Zealand) I could no longer walk, due to knee contractures. My hands resembled claws. I had elbow contractures and neck contractures, and could not straighten my arms... |
| Sherry: Fibromyalgia, Hypothyroidism and Raynaud's He said Fibromyalgia is just pain, and if I could not handle my job, I should quit... |
| Sue W: UCTD with Neurological and Musculoskeletal Involvement Prior to this unique onset of symptoms I worked as a nurse and I was a very independent, self-sufficient single parent... |
| Tina Lech: Diffuse Scleroderma, Fibromyalgia, Pineal Cyst I was a very active, alive, mother of three... |
| William M: Scleroderma or Lupus I had to give up my career of being a registered nurse (RN) over two years ago due to total disability... |
| Related Links |
| An autopsied case of Sjogren's syndrome with massive necrotic and demyelinating lesions of the cerebellar white matter. PubMed. J Neurol Sci. 2004 Oct 15;225(1-2):143-8. (Also see: Sjogren's Syndrome) |
| Brain abscesses caused by Abiotrophia defectiva: complication of immunosuppressive therapy in a patient with connective-tissue disease. PubMed. Scand J Infect Dis. 2004;36(6-7):497-9. (Also see: Medications, Lupus, Sjogren's Syndrome, and Overlap Syndrome |
| 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. |
| Scientists Build On Case Connecting Inflammatory Disease And Depression. Depressive disorders occur in 12 to 30 percent of patients who suffer from various diseases with an inflammatory component, including coronary heart disease and autoimmune diseases such as rheumatoid arthritis and inflammatory bowels. These mood disorders usually are attributed to psychological problems encountered by patients having to deal with the suffering and disability brought about by their diseases. However research on patients whose immune systems are boosted by immunotherapy challenge this view and suggest that depression may originate in the immune system. ScienceDaily 07/29/04. (Also see: Associated Conditions: Depression and Emotional Adjustment: Depression) |
| 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. |
