Diffuse Cerebral Sclerosis of Schilder
Multiple Sclerosis
Sclerosis
Amyotrophic Lateral Sclerosis
Tuberous Sclerosis
Cerebral Infarction
Sleep Deprivation
Seizures
Tablets
Electroencephalography
Epilepsy, Absence
Pamphlets
Proteolipoprotein gene analysis in 82 patients with sporadic Pelizaeus-Merzbacher Disease: duplications, the major cause of the disease, originate more frequently in male germ cells, but point mutations do not. The Clinical European Network on Brain Dysmyelinating Disease. (1/120)
Pelizaeus-Merzbacher Disease (PMD) is an X-linked developmental defect of myelination affecting the central nervous system and segregating with the proteolipoprotein (PLP) locus. Investigating 82 strictly selected sporadic cases of PMD, we found PLP mutations in 77%; complete PLP-gene duplications were the most frequent abnormality (62%), whereas point mutations in coding or splice-site regions of the gene were involved less frequently (38%). We analyzed the maternal status of 56 cases to determine the origin of both types of PLP mutation, since this is relevant to genetic counseling. In the 22 point mutations, 68% of mothers were heterozygous for the mutation, a value identical to the two-thirds of carrier mothers that would be expected if there were an equal mutation rate in male and female germ cells. In sharp contrast, among the 34 duplicated cases, 91% of mothers were carriers, a value significantly (chi2=9. 20, P<.01) in favor of a male bias, with an estimation of the male/female mutation frequency (k) of 9.3. Moreover, we observed the occurrence of de novo mutations between parental and grandparental generations in 17 three-generation families, which allowed a direct estimation of the k value (k=11). Again, a significant male mutation imbalance was observed only for the duplications. The mechanism responsible for this strong male bias in the duplications may involve an unequal sister chromatid exchange, since two deletion events, responsible for mild clinical manifestations, have been reported in PLP-related diseases. (+info)Embryonic stem cell-derived glial precursors: a source of myelinating transplants. (2/120)
Self-renewing, totipotent embryonic stem (ES) cells may provide a virtually unlimited donor source for transplantation. A protocol that permits the in vitro generation of precursors for oligodendrocytes and astrocytes from ES cells was devised. Transplantation in a rat model of a human myelin disease shows that these ES cell-derived precursors interact with host neurons and efficiently myelinate axons in brain and spinal cord. Thus, ES cells can serve as a valuable source of cell type-specific somatic precursors for neural transplantation. (+info)Identification of a new exon in the myelin proteolipid protein gene encoding novel protein isoforms that are restricted to the somata of oligodendrocytes and neurons. (3/120)
The myelin proteolipid protein (PLP) gene (i.e., the PLP/DM20 gene) has been of some interest because of its role in certain human demyelinating diseases, such as Pelizaeus-Merzbacher disease. A substantial amount of evidence, including neuronal pathology in knock-out and transgenic animals, suggests the gene also has functions unrelated to myelin structure, but the products of the gene responsible for these putative functions have not yet been identified. Here we report the identification of a new exon of the PLP/DM20 gene and at least two new products of the gene that contain this exon. The new exon, located between exons 1 and 2, is spliced into PLP and DM20 mRNAs creating a new translation initiation site that generates PLP and DM20 proteins with a 12 amino acid leader sequence. This leader sequence appears to target these proteins to a different cellular compartment within the cell bodies of oligodendrocytes and away from the myelin membranes. Furthermore, these new products are also expressed in a number of neuronal populations within the postnatal mouse brain, including the cerebellum, hippocampus, and olfactory system. We term these products somal-restricted PLP and DM20 proteins to distinguish them from the classic PLP and DM20 proteolipids. They represent putative candidates for some of the nonmyelin-related functions of the PLP/DM20 gene. (+info)Pathognomonic MR imaging findings in Balo concentric sclerosis. (4/120)
Irregular, concentric zones of increased signal on T2-weighted cranial MR imaging studies may strongly suggest Balo concentric sclerosis (BCS), a rare but recognized variant of multiple sclerosis. Differentiating BCS from multiple sclerosis or neoplasm can be difficult clinically, but MR imaging findings noted in this case may be pathognomonic. (+info)Balo's concentric sclerosis: clinical and radiologic features of five cases. (5/120)
Balo's concentric sclerosis (BCS) is a rare demyelinating disease considered to be a variant of multiple sclerosis. Five BCS cases were diagnosed antemortem based on their typical concentric mass patterns on MR images and based on clinical and CSF findings. Histopathologic investigation was also performed in one case. Our case report supports the concept that BCS may be a self-limited disease that is not always fatal. Characteristic MR imaging findings may allow antemortem diagnosis of BCS when performed at the onset of the disease. (+info)MR spectroscopic findings in a case of Alpers-Huttenlocher syndrome. (6/120)
Alpers-Huttenlocher syndrome, considered a mitochondrial disease, combines encephalopathy and liver failure. An 11-year-old boy with Alpers-Huttenlocher syndrome underwent conventional MR imaging, diffusion-weighted imaging, and proton MR spectroscopy. Diffusion-weighted imaging showed cytotoxic edema interpreted as acute-phase encephalopathy. MR spectroscopy revealed a lactate peak in the cortex that appeared abnormal on diffusion-weighted images, possibly representing respiratory deficiency with anaerobic metabolism. MR spectroscopy proved to be more sensitive regarding lactate detection than did neurometabolic examination of serum and CSF. A reduced N-acetylaspartate-creatine ratio was detected in both the cortex that appeared abnormal and the cortex that appeared normal on the diffusion-weighted images, indicating neuronal damage that was widespread, even beyond the boundaries of conventional MR imaging changes. (+info)Demyelinization induced in the brains of monkeys by means of fast neutrons; pathogenesis of the lesion and comparison with the lesions of multiple sclerosis and Schilder's disease. (7/120)
Demyelinization was regularly conspicuous in the white matter of the rostral portions of the brains of 6 monkeys sacrificed 14 to 22 months after exposure of the ocular regions to 850 r.e.p. of 14 mev. neutron radiation and it was not present in the brain of a monkey 2 months after radiation under identical conditions; or in those of 5 non-radiated animals serving as controls. In early lesions, the individual myelin sheaths were varicose and fragmented, while the neurons, axons, and glial cells remained normal in appearance. With the passage of time, the degeneration of myelin became more marked and in later stages was accompanied by a degeneration of the axis cylinders, a proliferation of astrocytes and microglia, and minor cytological changes in the oligodendroglia, the whole process occurring essentially without inflammation or notable changes in the cerebral or meningeal blood vessels. The findings show that neutron radiation has the property of destroying myelin in the living animal and inducing changes that are notably similar in their pathogenesis to those that characterize disseminated encephalomyelitis in human beings. (+info)Devic's neuromyelitis optica and Schilder's myelinoclastic diffuse sclerosis. (8/120)
An adult patient developed both Devic's neuromyelitis optica and Schilder's myelinoclastic diffuse sclerosis, suggesting that these entities represent rare topographical and aggressive variants within the spectrum of multiple sclerosis. (+info)Diffuse cerebral sclerosis of Schilder, also known as Schilder's disease, is a rare inflammatory demyelinating disorder of the central nervous system. It primarily affects children and young adults, but can occur at any age. The condition is characterized by widespread destruction of the myelin sheath, which surrounds and protects nerve fibers in the brain.
The hallmark feature of Schilder's disease is the presence of multiple, large, symmetrical lesions in the white matter of both cerebral hemispheres. These lesions are typically located in the parieto-occipital regions of the brain and can extend to involve other areas as well.
The symptoms of Schilder's disease vary depending on the location and extent of the lesions, but may include:
* Progressive intellectual decline
* Seizures
* Visual disturbances
* Weakness or paralysis on one side of the body (hemiparesis)
* Loss of sensation in various parts of the body
* Speech difficulties
* Behavioral changes, such as irritability, mood swings, and depression
The exact cause of Schilder's disease is not known, but it is believed to be an autoimmune disorder, in which the body's own immune system mistakenly attacks the myelin sheath. There is no cure for Schilder's disease, and treatment typically involves corticosteroids or other immunosuppressive therapies to reduce inflammation and slow the progression of the disease. Despite treatment, many patients with Schilder's disease experience significant disability and may require long-term care.
Multiple Sclerosis (MS) is a chronic autoimmune disease that affects the central nervous system (CNS), which includes the brain, spinal cord, and optic nerves. In MS, the immune system mistakenly attacks the protective covering of nerve fibers, called myelin, leading to damage and scarring (sclerosis). This results in disrupted communication between the brain and the rest of the body, causing a variety of neurological symptoms that can vary widely from person to person.
The term "multiple" refers to the numerous areas of scarring that occur throughout the CNS in this condition. The progression, severity, and specific symptoms of MS are unpredictable and may include vision problems, muscle weakness, numbness or tingling, difficulty with balance and coordination, cognitive impairment, and mood changes. There is currently no cure for MS, but various treatments can help manage symptoms, modify the course of the disease, and improve quality of life for those affected.
Sclerosis is a medical term that refers to the abnormal hardening or scarring of body tissues, particularly in the context of various degenerative diseases affecting the nervous system. The term "sclerosis" comes from the Greek word "skleros," which means hard. In these conditions, the normally flexible and adaptable nerve cells or their protective coverings (myelin sheath) become rigid and inflexible due to the buildup of scar tissue or abnormal protein deposits.
There are several types of sclerosis, but one of the most well-known is multiple sclerosis (MS). In MS, the immune system mistakenly attacks the myelin sheath surrounding nerve fibers in the brain and spinal cord, leading to scarring and damage that disrupts communication between the brain and the rest of the body. This results in a wide range of symptoms, such as muscle weakness, numbness, vision problems, balance issues, and cognitive impairment.
Other conditions that involve sclerosis include:
1. Amyotrophic lateral sclerosis (ALS): Also known as Lou Gehrig's disease, ALS is a progressive neurodegenerative disorder affecting motor neurons in the brain and spinal cord, leading to muscle weakness, stiffness, and atrophy.
2. Systemic sclerosis: A rare autoimmune connective tissue disorder characterized by thickening and hardening of the skin and internal organs due to excessive collagen deposition.
3. Plaque psoriasis: A chronic inflammatory skin condition marked by red, scaly patches (plaques) resulting from rapid turnover and accumulation of skin cells.
4. Adhesive capsulitis: Also known as frozen shoulder, this condition involves stiffening and thickening of the shoulder joint's capsule due to scarring or inflammation, leading to limited mobility and pain.
Cerebral arteries refer to the blood vessels that supply oxygenated blood to the brain. These arteries branch off from the internal carotid arteries and the vertebral arteries, which combine to form the basilar artery. The major cerebral arteries include:
1. Anterior cerebral artery (ACA): This artery supplies blood to the frontal lobes of the brain, including the motor and sensory cortices responsible for movement and sensation in the lower limbs.
2. Middle cerebral artery (MCA): The MCA is the largest of the cerebral arteries and supplies blood to the lateral surface of the brain, including the temporal, parietal, and frontal lobes. It is responsible for providing blood to areas involved in motor function, sensory perception, speech, memory, and vision.
3. Posterior cerebral artery (PCA): The PCA supplies blood to the occipital lobe, which is responsible for visual processing, as well as parts of the temporal and parietal lobes.
4. Anterior communicating artery (ACoA) and posterior communicating arteries (PComAs): These are small arteries that connect the major cerebral arteries, forming an important circulatory network called the Circle of Willis. The ACoA connects the two ACAs, while the PComAs connect the ICA with the PCA and the basilar artery.
These cerebral arteries play a crucial role in maintaining proper brain function by delivering oxygenated blood to various regions of the brain. Any damage or obstruction to these arteries can lead to serious neurological conditions, such as strokes or transient ischemic attacks (TIAs).
Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disorder that affects nerve cells in the brain and spinal cord responsible for controlling voluntary muscle movements, such as speaking, walking, breathing, and swallowing. The condition is characterized by the degeneration of motor neurons in the brain (upper motor neurons) and spinal cord (lower motor neurons), leading to their death.
The term "amyotrophic" comes from the Greek words "a" meaning no or negative, "myo" referring to muscle, and "trophic" relating to nutrition. When a motor neuron degenerates and can no longer send impulses to the muscle, the muscle becomes weak and eventually atrophies due to lack of use.
The term "lateral sclerosis" refers to the hardening or scarring (sclerosis) of the lateral columns of the spinal cord, which are primarily composed of nerve fibers that carry information from the brain to the muscles.
ALS is often called Lou Gehrig's disease, named after the famous American baseball player who was diagnosed with the condition in 1939. The exact cause of ALS remains unknown, but it is believed to involve a combination of genetic and environmental factors. There is currently no cure for ALS, and treatment primarily focuses on managing symptoms and maintaining quality of life.
The progression of ALS varies from person to person, with some individuals experiencing rapid decline over just a few years, while others may have a more slow-progressing form of the disease that lasts several decades. The majority of people with ALS die from respiratory failure within 3 to 5 years after the onset of symptoms. However, approximately 10% of those affected live for 10 or more years following diagnosis.
Tuberous Sclerosis Complex (TSC) is a rare genetic disorder that causes non-cancerous (benign) tumors to grow in many parts of the body. These tumors can affect the brain, skin, heart, kidneys, eyes, and lungs. The signs and symptoms of TSC can vary widely, depending on where the tumors develop and how severely a person is affected.
The condition is caused by mutations in either the TSC1 or TSC2 gene, which regulate a protein that helps control cell growth and division. When these genes are mutated, the protein is not produced correctly, leading to excessive cell growth and the development of tumors.
TSC is typically diagnosed based on clinical symptoms, medical imaging, and genetic testing. Treatment for TSC often involves a multidisciplinary approach, with specialists in neurology, dermatology, cardiology, nephrology, pulmonology, and ophthalmology working together to manage the various symptoms of the condition. Medications, surgery, and other therapies may be used to help control seizures, developmental delays, skin abnormalities, and other complications of TSC.
Cerebral infarction, also known as a "stroke" or "brain attack," is the sudden death of brain cells caused by the interruption of their blood supply. It is most commonly caused by a blockage in one of the blood vessels supplying the brain (an ischemic stroke), but can also result from a hemorrhage in or around the brain (a hemorrhagic stroke).
Ischemic strokes occur when a blood clot or other particle blocks a cerebral artery, cutting off blood flow to a part of the brain. The lack of oxygen and nutrients causes nearby brain cells to die. Hemorrhagic strokes occur when a weakened blood vessel ruptures, causing bleeding within or around the brain. This bleeding can put pressure on surrounding brain tissues, leading to cell death.
Symptoms of cerebral infarction depend on the location and extent of the affected brain tissue but may include sudden weakness or numbness in the face, arm, or leg; difficulty speaking or understanding speech; vision problems; loss of balance or coordination; and severe headache with no known cause. Immediate medical attention is crucial for proper diagnosis and treatment to minimize potential long-term damage or disability.
Sleep deprivation is a condition that occurs when an individual fails to get sufficient quality sleep or the recommended amount of sleep, typically 7-9 hours for adults. This can lead to various physical and mental health issues. It can be acute, lasting for one night or a few days, or chronic, persisting over a longer period.
The consequences of sleep deprivation include:
1. Fatigue and lack of energy
2. Difficulty concentrating or remembering things
3. Mood changes, such as irritability or depression
4. Weakened immune system
5. Increased appetite and potential weight gain
6. Higher risk of accidents due to decreased reaction time
7. Health problems like high blood pressure, diabetes, and heart disease over time
Sleep deprivation can be caused by various factors, including stress, shift work, sleep disorders like insomnia or sleep apnea, poor sleep hygiene, and certain medications. It's essential to address the underlying causes of sleep deprivation to ensure proper rest and overall well-being.
A seizure is an uncontrolled, abnormal firing of neurons (brain cells) that can cause various symptoms such as convulsions, loss of consciousness, altered awareness, or changes in behavior. Seizures can be caused by a variety of factors including epilepsy, brain injury, infection, toxic substances, or genetic disorders. They can also occur without any identifiable cause, known as idiopathic seizures. Seizures are a medical emergency and require immediate attention.
In the context of medical terminology, tablets refer to pharmaceutical dosage forms that contain various active ingredients. They are often manufactured in a solid, compressed form and can be administered orally. Tablets may come in different shapes, sizes, colors, and flavors, depending on their intended use and the manufacturer's specifications.
Some tablets are designed to disintegrate or dissolve quickly in the mouth, making them easier to swallow, while others are formulated to release their active ingredients slowly over time, allowing for extended drug delivery. These types of tablets are known as sustained-release or controlled-release tablets.
Tablets may contain a single active ingredient or a combination of several ingredients, depending on the intended therapeutic effect. They are typically manufactured using a variety of excipients, such as binders, fillers, and disintegrants, which help to hold the tablet together and ensure that it breaks down properly when ingested.
Overall, tablets are a convenient and widely used dosage form for administering medications, offering patients an easy-to-use and often palatable option for receiving their prescribed treatments.
Electroencephalography (EEG) is a medical procedure that records electrical activity in the brain. It uses small, metal discs called electrodes, which are attached to the scalp with paste or a specialized cap. These electrodes detect tiny electrical charges that result from the activity of brain cells, and the EEG machine then amplifies and records these signals.
EEG is used to diagnose various conditions related to the brain, such as seizures, sleep disorders, head injuries, infections, and degenerative diseases like Alzheimer's or Parkinson's. It can also be used during surgery to monitor brain activity and ensure that surgical procedures do not interfere with vital functions.
EEG is a safe and non-invasive procedure that typically takes about 30 minutes to an hour to complete, although longer recordings may be necessary in some cases. Patients are usually asked to relax and remain still during the test, as movement can affect the quality of the recording.
Absence epilepsy is a type of epilepsy characterized by recurrent brief episodes of "absences," or staring spells, that can last from a few seconds to several minutes. These episodes are often accompanied by subtle body movements such as lip smacking or eyelid flutters. Absence epilepsy is most commonly diagnosed in children and adolescents, and it is more common in girls than boys.
The seizures in absence epilepsy are caused by abnormal electrical activity in the brain, specifically in a part of the brain called the cortex. These abnormal electrical discharges occur in a pattern that involves both sides of the brain simultaneously. This differs from other types of epilepsy, which may involve only one side of the brain or specific areas within a single hemisphere.
Absence seizures are typically brief and do not cause confusion or disorientation after they end. However, if they occur frequently, they can interfere with learning and social development. In some cases, absence epilepsy may be associated with other types of seizures, such as generalized tonic-clonic (grand mal) seizures or myoclonic jerks.
The diagnosis of absence epilepsy is usually made based on the characteristic symptoms and the results of an electroencephalogram (EEG), which can detect the abnormal electrical activity in the brain during a seizure. Treatment typically involves medication to control the seizures, such as ethosuximide or valproic acid. In some cases, a ketogenic diet may also be recommended as an alternative treatment option.
I'm sorry for any confusion, but "pamphlets" is not a medical term. It refers to a small paper booklet or leaflet that can be used to provide information on various topics, including non-medical subjects. If you have any questions about medical terminology or concepts, I'd be happy to help with those!
Neurology is a branch of medicine that deals with the study and treatment of diseases and disorders of the nervous system, which includes the brain, spinal cord, peripheral nerves, muscles, and autonomic nervous system. Neurologists are medical doctors who specialize in this field, diagnosing and treating conditions such as stroke, Alzheimer's disease, epilepsy, Parkinson's disease, multiple sclerosis, and various types of headaches and pain disorders. They use a variety of diagnostic tests, including imaging studies like MRI and CT scans, electrophysiological tests like EEG and EMG, and laboratory tests to evaluate nerve function and identify any underlying conditions or abnormalities. Treatment options may include medication, surgery, rehabilitation, or lifestyle modifications.
List of MeSH codes (C20)
List of MeSH codes (C10)
CNS demyelinating autoimmune diseases
Inflammatory demyelinating diseases of the central nervous system
Lesional demyelinations of the central nervous system
Spongy degeneration of the central nervous system
List of neurological conditions and disorders
Multiple sclerosis
List of skin conditions
Diffuse Cerebral Sclerosis of Schilder | Profiles RNS
Alpers-Huttenlocher syndrome: MedlinePlus Genetics
Diffuse Sclerosis: Background, Pathophysiology, Epidemiology
Megalencephalic leukoencephalopathy with subcortical cysts | Radiology Reference Article | Radiopaedia.org
Krabbe disease | Radiology Reference Article | Radiopaedia.org
List of MeSH codes (C20) - Wikipedia
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Myelinoclastic diffuse sclerosis presenting as a mass lesion in a child with turner's syndrome<...
Diffuse Sclerosis: Background, Pathophysiology, Epidemiology
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Schilder's Disease1
- A rare case of myelinoclastic diffuse sclerosis (MDS), occasionally referred to as Schilder's disease, is reported in a child with Turner's syndrome. (elsevierpure.com)
Acute disseminated ence3
- Some have suggested that this child had either acute multiple sclerosis or, less likely, severe acute disseminated encephalomyelitis . (medscape.com)
- The distribution of lesions in this index case (ie, bilateral but slightly asymmetrical large sharply demarcated areas of demyelination) and the resemblance of the pathology to multiple sclerosis (which in turn resembles the pathology of acute disseminated encephalomyelitis) became the hallmarks of the diagnosis of Schilder disease. (medscape.com)
- The fact that a large number of pathologically diagnosed cases have proven on reanalysis to be examples of diseases other than Schilder disease supports the view that many of these radiologically diagnosed cases are also something other than Schilder disease, such as encephalitis, acute disseminated encephalomyelitis, acute multiple sclerosis, leukodystrophy, SSPE, mitochondrial cytopathies, or other possibilities. (medscape.com)
Adrenoleukodystrophy1
- This information has permitted the diagnosis of most of these cases to be reassigned to other diseases such as tumor, multiple sclerosis, adrenoleukodystrophy, encephalitis, or subacute sclerosing panencephalitis (SSPE). (medscape.com)
Alpers2
- Alpers' Syndrome refers to a heterogeneous group of diseases that feature progressive cerebral deterioration and liver disease. (sdsu.edu)
- Alpers disease consists of diffuse cerebral degeneration manifested as developmental delay, seizures, vomiting, and progressive neuromuscular deterioration, with liver disease and death. (omeka.net)
Diseases2
- They are sometimes considered different diseases from Multiple Sclerosis, [4] [5] but considered by others to form a spectrum differing only in terms of chronicity, severity, and clinical course. (mdwiki.org)
- Some anti- neurofascin demyelinating diseases were previously considered a subtype of Multiple Sclerosis but now they are considered a separate entity, as it happened before to anti-MOG and anti-AQP4 cases. (mdwiki.org)
Degeneration2
- In some of these cases, the diagnosis of Schilder disease was based on pathological analysis of the brains of patients who had died from brain degeneration. (medscape.com)
- Megalencephalic leukoencephalopathy with subcortical cysts , also known as Van der Knaap disease , refers to a rare inherited autosomal recessive disease characterized by diffuse subcortical leukoencephalopathy associated with white matter cystic degeneration. (radiopaedia.org)
Myelinoclastic1
- abstract = "A rare case of myelinoclastic diffuse sclerosis (MDS), occasionally referred to as Schilder{\textquoteright}s disease, is reported in a child with Turner{\textquoteright}s syndrome. (elsevierpure.com)
Pathologic3
- Pathologic findings include a large, sharply defined, asymmetric focus of myelin destruction that may involve an entire lobe or cerebral hemisphere. (sdsu.edu)
- Concentric sclerosis of Balo is differentiated from diffuse cerebral sclerosis of Schilder by the pathologic finding of alternating bands of destruction and preservation of myelin in concentric rings. (sdsu.edu)
- The pathologic features are similar to MULTIPLE SCLEROSIS , including perivascular and periventricular foci of inflammation and demyelination. (nih.gov)
Syndrome2
- Paul Schilder described a severe and fulminating syndrome of acute demyelinating disease in 1912. (medscape.com)
- Multiple sclerosis for some people is a syndrome more than a single disease. (mdwiki.org)
Disease9
- Her neurologic disease followed a course of 4.5 months of deterioration leading to death and ensuing pathological examination of her brain by Schilder. (medscape.com)
- The disease mechanisms and the pathological features of these conditions were unknown at the time that Schilder published his reports. (medscape.com)
- The availability of pathological descriptions has permitted many cases to be carefully reconsidered with the benefit of subsequent knowledge of pathological entities unknown to Schilder and to many of the subsequent individuals who have published cases of suspected Schilder disease. (medscape.com)
- Based upon the appearance of lesions in these imaging studies, a number of cases of supposed Schilder disease have been diagnosed in living individuals, without benefit of pathological analysis of the brain or a brain biopsy specimen. (medscape.com)
- Many of these individuals with radiographically diagnosed disease have survived much longer than has tended to be the case in individuals whose Schilder disease was diagnosed on the basis of postmortem pathological analysis. (medscape.com)
- Whether such cases represent examples less fulminant forms of Schilder disease or whether postmortem pathological confirmation selects for the most fulminant and lethal forms of Schilder disease is unclear. (medscape.com)
- Lewis HS, Willis E, Veerapandiyan A. Charcot-Marie-Tooth Disease Type 4J and Multiple Sclerosis. (uams.edu)
- Lewy body (ies) (disease) (G31.8) · multiple sclerosis (G35) · neurosyphilis (A52.1) · niacin deficiency [pellagra] (E52) · polyarteritis nodosa (M30.0) · systemic lupus erythematosus (M32. (who.int)
- BACKGROUND: In relapsing-remitting multiple sclerosis (RRMS), smoking is a known risk factor for disease susceptibility and disability progression. (bvsalud.org)
Demyelination1
- Schilder found 2 large lesions that manifested demyelination with axon sparing. (medscape.com)
Multiple4
- The brain also contained other smaller lesions with a pathology typical for multiple sclerosis . (medscape.com)
- Ghadge VV, Bondre AG, Parate TR, Bhiwgade RD. Marburg variant of multiple sclerosis: A case report. (vjim.org)
- Multiple sclerosis (MS) is a demyelinating disorder with central nervous system (CNS) involvement, leading to varied presentations. (vjim.org)
- Anti-MOG associated spectrum , often clinically presented as an anti- MOG autoimmune encephalomyelitis , [21] [22] but can also appear as negative NMO or atypical multiple sclerosis. (mdwiki.org)
Patients2
- Subsequent reports by Schilder (1913 and 1924) included 2 additional patients, each with pathological changes that differed from the other and from the original case. (medscape.com)
- Macrophages expressing C-C chemokine receptor type 2 (CCR2) infiltrate the central and peripheral neural tissues of amyotrophic lateral sclerosis (ALS) patients. (bvsalud.org)
People2
- This graph shows the total number of publications written about "Diffuse Cerebral Sclerosis of Schilder" by people in this website by year, and whether "Diffuse Cerebral Sclerosis of Schilder" was a major or minor topic of these publications. (sdsu.edu)
- Below are the most recent publications written about "Diffuse Cerebral Sclerosis of Schilder" by people in Profiles. (sdsu.edu)
Myelinoclastic diffuse4
- Pathological analysis by autopsy or biopsy demonstrates histologic changes consistent with subacute or chronic myelinoclastic diffuse sclerosis, changes which in essence cannot be distinguished from those of multiple sclerosis. (medscape.com)
- Jarius S, Haas J, Paul F, Wildemann B. Myelinoclastic diffuse sclerosis (Schilder's disease) is immunologically distinct from multiple sclerosis: results from retrospective analysis of 92 lumbar punctures. (medscape.com)
- Poser CM. Myelinoclastic diffuse sclerosis. (medscape.com)
- Kotil K, Kalayci M, Köseoglu T, Tugrul A. Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature. (medscape.com)
SCHILDER'S DISEASE1
- Computerised tomography findings in multiple sclerosis and Schilder's disease. (medscape.com)
Periaxialis diffusa2
- Schilder P. Zur Frage der Encephalitis periaxialis diffusa. (medscape.com)
- Schilder P. Die Encephalitis periaxialis diffusa. (medscape.com)
Progression3
- Complications include cerebral herniation, inexorable progression of disease to death, development of pneumonia, sepsis, pulmonary embolization, skin breakdown and ulceration in individuals who are moribund, and the various complications due to corticosteroid administration. (medscape.com)
- Baló concentric sclerosis is considered by some authors to be a variant of Schilder disease, with MRI lesions showing a characteristic alternating pattern of spared and damaged white matter that suggests progression of the disease process from the ventricles outward. (medscape.com)
- Baló concentric sclerosis is often associated with more inflammatory cerebrospinal fluid (CSF) findings and a more fulminant progression than typical MS. (medscape.com)
Neuronal1
- Neuropathologic changes are most severe in the gray matter and consist of diffuse neuronal loss, spongiform changes, and astrocytosis. (nih.gov)
Diagnostic1
- Dupel-Pottier C. [Diagnostic criteria of borderline forms of multiple sclerosis]. (medscape.com)
Clinical1
- Based upon the 9 cases that have satisfied Poser's criteria, the following statements can be made concerning the history that precedes clinical presentation of Schilder disease. (medscape.com)
Progressive1
- In 1897 Heubner described a young boy with rapidly progressive neurologic deterioration consistent with X-ALD, classified as having "diffuse sclerosis" on autopsy [ 10 ]. (biomedcentral.com)
Sklerose2
- Schilder P. Zur Kenntnis der sogenannten diffusen Sklerose. (medscape.com)
- Bielschowsky M, Henneberg R. Uber familiare diffuse Sklerose (Leukodystrophia cerebri progressiva hereditaria). (medscape.com)
Unclear1
- Although note evidence for a possible infectious illness at the onset of presumed Schilder disease, the significance of this observation remains unclear. (medscape.com)
19101
- Cases of "diffuse sclerosis" that resemble X-ALD were also described in 1899 by Ceni [ 11 ] and in 1910 by Haberfield and Spieler [ 12 ]. (biomedcentral.com)
Characteristic1
- No features of the general examination are characteristic features of the presentation of Schilder disease. (medscape.com)
Onset2
- In some reports, latency exists between this initial febrile illness and the subacute onset of Schilder disease. (medscape.com)
- Other cases have a fulminant course without such clear distinction between prodrome and onset of the disease process thought to be Schilder disease. (medscape.com)