Wednesday, 18 July 2018

Progressive Supranuclear Palsy (PSP)

Progressive Supranuclear Palsy (PSP) is caused by damage to certain neurons that results in progressive lack of coordination, stiffness in neck and trunk, slow movements, difficulty with eye movement, difficulty walking which may result in falls and cognitive dysfunctions. The disorder is found commonly in people above 60 years of age.
The disorder is also called as Steele- Richardson- Olszewsky syndrome

The classical symptom of PSP  involve a tendency to fall unexpectedly, usually backwards. Other common symptoms include rigidity and backward arching of the neck, and a key diagnostic feature, the Supranuclear Palsy. This is a difficulty in ‘willed’ upgaze and downgaze, ie. the ability of the patient to voluntarily move their eyes up and down while keeping the head still.

Since the symptoms of PSP are similar to Parkinson's disease, the disorder could be misdiagnosed as Parkinson's in its early stages. 
Motor symptoms come first and always precede cognitive changes.

The cause of PSP is as yet unknown, though there may be a genetic, as well as an environmental, component. However, as observed in many neurodegenerative disorders, tau proteins or tau gene is likely a significant causal factor. Indeed, there is a genetic risk factor linked to H1H1 haplotype in the tau gene.
Source: https://goo.gl/7L87DP


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Tuesday, 10 July 2018

Chronic Inflammatory Demyelinating Polyneuropathy

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an immune mediated inflammatory neurological disorder. The disease damages the protective myelin sheath of the peripheral nerves. This results in the progressive weakness and loss of sensation in the legs and arms. The disease is closely related to Guillain- Barre syndrome and is the chronic counter part of the acute disease. 
CIDP is also called as chronic relapsing polyneuropathy.

The disease affects the young adults and is more in men than women. The symptoms may include tingling or numbness in the toes or fingers, weakness of legs and arms, fatigue and abnormal sensation.

The disease can be treated with corticosteroids like prednisone, which may be given alone or in combination with immunosuppressants. Plasmapheresis or intravenous immunoglobulin can be an effective therapy. Physiotherapy can be done to minimize the shrinkage of muscles, improve the strength, function and mobility.

The causes of CIDP varies widely among the individuals. CIDP can be treatable and the early diagnosis of the condition can help minimizing the axonal loss and improves functional recovery.


Saturday, 7 July 2018

Metachromatic Leukodystrophy (MLD)

Metachromatic Leukodystrophy (MLD) is a autosomal recessive disorder of myelin metabolism. The disease is characteriseed by the deficiency of arylsufatase enzyme which causes sulfatides to get deposited in the white matter of central and peripheral nervous system and other organs like kidney, liver, pancreas, testes and retina.
The sulfatides show peuliar staining called, metachromatic.

The onset of adult variant MLD may ocuur in late teens to the seventh decade of a person. The signs and symptoms may include personality and behavioral changes and intellectual deterioration. The patients may experience memory and concentration impairment and may become childish. The disease may be misdiagnosed of schizophrenia due to its atypical psychotic features. In many cases, peripheral neuropathy may be the common presenting feature. The disease progresses slowly and the survival time is 14 years. 

The cause of the disease is the mutation in the arylsufatase A gene which results in the abnormal storage of sulfatides. There may be diffuse demyelinisation in the white matter of the central nervous system.



Wednesday, 4 July 2018

Neuroinformatics


Neuroinformatics is a combination of neuroscience and information science. It deals with the organisation of neuroscience data and knowledge bases of nervous system and application of tools and computational models for data analysis, acquisition, visualization and distribution. 
Brain mapping helps the scientists to know what the brain does and how various parts of the brain work. Brain mapping is a set of neuroscience techniques used to view the structural and functional aspects of the brain onto a spatial representation called maps. Due to the advancements and research in the field of neuroinformatics, large amount of data is being analysed and interpreted using various tools.
Many techniques for visualization and analysis of data have been developed by other fields. But the neuroscience community is in need of special-purpose, optimized tools and algorithms. Data only make sense in the context of tools. Navigation and manipulation of data requires a multitude of tools. Integration builds not only on the accumulation of data within and across the many levels of investigation, but also on the tools used to compare data, create higher order representations, and extract principles. Over time, some of these tools may even benefit researchers in branches of the information sciences as they deal with issues related to brain function such as machine learning and robotic task planning.
To share and gain knowledge on the various aspects of neuroinformatics, join the "7th International Conference on Neurology and  Neuromuscular Diseases" that will be held on October 22-23, 2018 at Madrid, Spain. To know more, visit: https://bit.ly/2I9Frf7Drop a mail to: neuromuscular@pulsusevents.org




Tuesday, 3 July 2018

Viruses as an effective immune therapy to treat brain cancer

A study by researchers have shown that viruses were effective in the treatment of brain cancer by killing the brain cells. 

The researchers have found that a type of virus called, reovirus was able to cross the protective blood- brain barrier of the brain and replicate and attack the cancer cells of brain cancer and cancers that have spread to the brain. They have also reported that the reovirus is able to switch on the body's immune system to attack the cancer.

This naturally occurring virus could be injected directly into the blood stream and can acts as an effective immune therapy in patients with aggressive brain cancer and cancers that have spread to the brain.
source: https://goo.gl/XY7eti

To share and gain knowledge on the various aspects of brain cancer, join the "7th International Conference on Neurology and  Neuromuscular Diseases" that will be held on October 22-23, 2018 at Madrid, Spain. To know more, visit: https://bit.ly/2I9Frf7





Saturday, 30 June 2018

Adrenoleukodystrophy (ALD)

Adrenoleukodystrophy (ALD) is a X- chromosomal recessive disease that leads to adrenal gland dysfunction. The disease is mainly characterized by abnormal storage of very long chain fatty acids in myelin and also in other cells of the body. A variant of the disease is adrenomyeloneuropathy (AMN), which mainly affects the spinal cord and peripheral nerves. This results in spastic paraparesis, anal sphincter dysfunction and abnormalities in legs and bladder.

The disease onsets at the age of 25- 30 years. The clinical features of the disease includes, behavioral disorders, psychotic symptoms, impaired sexual function, ataxia, dyskinesia, polyneuropathy, etc. The neurological and psychiatric symptoms are accompanied by adrenal gland dysfunction and hypogonadism.

ALD is caused by mutations in the ABCD1 gene (Xq28), which encodes a transporter involved in the import of very long-chain fatty acids into the peroxisome. The storage of abnormally long fatty acids alters the properties of myelin and results in demyelinisation.




Thursday, 28 June 2018

Multiple System Atropy

Multiple System Atropy (MSA) is a progressive neurodegenerative disorder of the central and sympathetic nervous system and is characterized by postural or orthostatic hypotension, that is an excessive drop in the blood pressure when the person stands up. This causes dizziness and momentary blackouts. 
MSA does not cause dementia but it may impair other cognitive functions. 

MSA is classified into three types namely, olivopontocerebellar atropy (OPCA), which affects balance, coordination and speech; parkinsonian form called striatonigral degeneration, that resembles Parkinson's disease due to stiffness in muscles and impaired movement; a mixed cerebellar and parkinsonian form. In all three types, patients may experience orthostatic hypotension. 
Constipation, impotence in men and urinary incontinence are predominantly seen early in the course of disease. 

The disorder is also known as Shy- Drager syndrome, and the disease can be difficult to diagnose in the early stages. The blood pressure in patients may be very less when they stand up and may be high when they lie down. Other symptoms include, difficulty in breathing and swallowing, impaired speech and inability to sweat. 
Cardiopulmonary arrest may be the reason for death in patients with breathing difficulties. 

The syndrome ends in death in 7 to 10 years after the diagnosis.
Orthostatic hypotension in Shy-Drager syndrome is treatable, but there is not known effective treatment for the progression central nervous system degeneration.

To discuss on more enthralling subjects like the above, join the "7th International Conference on Neurology and Neuromuscular Diseases" that will be held in October 22-23, 2018 at Madrid, Spain. Visit: https://neuromuscular.pulsusconference.com/
Drop us a mail to: neuromuscular@pulsusevents.org