Brain & Nervous System Disorders

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• QWhat is prosopagnosia?

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  A Diana Meeks , Family Medicine, answered on behalf of Sigma Nursing

  Prosopagnosia is a neurological disorder characterized by the inability to recognize faces. Prosopagnosia is also known as face blindness or facial agnosia. The term "prosopagnosia" comes from the Greek words for "face" and "lack of knowledge." Depending on the degree of impairment, some people with prosopagnosia may only have difficulty recognizing a familiar face, others will be unable to discriminate between unknown faces, and some may not even be able to distinguish a face as being different from an object. Some people with the disorder are unable to recognize their own face. Prosopagnosia is not related to memory dysfunction, memory loss, impaired vision, or learning disabilities. It is thought to be the result of abnormalities, damage, or impairment in the right fusiform gyrus, which is a fold in the brain that appears to coordinate the neural systems that control facial perception and memory. Prosopagnosia can result from a stroke, a traumatic brain injury, or certain neurodegenerative diseases. In some cases, prosopagnosia is a congenital disorder, present at birth in the absence of any brain damage. Congenital prosopagnosia appears to run in families, which makes it likely to be the result of a genetic mutation or deletion. Some degree of prosopagnosia is often present in children with autism and Asperger's syndrome and may be the cause of their impaired social development.

  This answer is based on source information from the National Institute of Neurological Disorders and Stroke.

• QWhat is microcephaly?

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  A Riverside Center for Neurosciences answered

  Microcephaly is a medical condition in which the circumference of the head is smaller than normal because the brain has not developed properly, or has stopped growing. Microcephaly can be present at birth or it may develop in the first few years of life. It is most often caused by genetic abnormalities that interfere with the growth of the cerebral cortex during the early months of fetal development. It is associated with Down's syndrome, chromosomal syndromes and neurometabolic syndromes. Babies may also be born with microcephaly if, during pregnancy, their mother abused drugs or alcohol; became infected with a cytomegalovirus, rubella (German measles), varicella (chicken pox) virus or possibly Zika virus; was exposed to certain toxic chemicals; or had untreated phenylketonuria (PKU). Babies born with microcephaly will have a smaller than normal head that will fail to grow as they progress through infancy.

  With viral-induced brain injury, such as with the Zika virus, there is often widespread tissue and cell death leading to brain shrinkage rather than simply impaired growth. The Zika virus is also associated with retinal lesions in about a third of cases, often leading to blindness.

  Depending on the severity of the accompanying syndrome, children with microcephaly may have mental retardation, delayed motor functions and speech, facial distortions, dwarfism or short stature, hyperactivity, seizures, difficulties with coordination and balance, and other brain or neurological abnormalities. Some children with microcephaly will have normal intelligence and a head that will grow bigger, but they will track below the normal growth curves for head circumference.

  This answer is based on source information from the National Institute of Neurological Disorders and Stroke.

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• QWhich children are good candidates for moyamoya surgery?

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  A UCLA Health answered

  Children are good candidates for moyamoya surgery if they have symptoms attributable to moyamoya and angiographic findings suggestive of progressive moyamoya. These findings include a Suzuki grade 2 angiographic appearance or "ivy sign" on a magnetic resonance imaging (MRI).

• QWhy must hypothalamic dysfunction be treated with multiple interventions?

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  A Dr. Kent A. Holtorf, MD , Endocrinology Diabetes & Metabolism, answered

  Although a concept that is sometimes uncomfortable and foreign to traditional medical styles of thinking, the need for multiple interventions is required for effective treatment of an illness that affects a critical control center (such as the hypothalamus), which impacts multiple systems. Unfortunately, there is not a single treatment that reverses hypothalamic dysfunction directly. Thus, this situation is different from illnesses that affect a single target organ and which can be treated with a single intervention. For example, pituitary dysfunction itself often requires treatment with several hormones. This effect is multiplied in hypothalamic dysfunction, which affects several critical systems in addition to the pituitary gland.
• QWhat causes ataxia?

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  A NewYork-Presbyterian Hospital answered

  Ataxia is usually caused by dysfunction of the cerebellum, the region of the brain where sensory perception, motor control, and voluntary movement are all integrated. Many people develop ataxia as the main symptom of more than two dozen inherited disorders, among them Friedreich's ataxia, spinocerebellar ataxias including Machado-Joseph disease, episodic ataxia, and fragile X tremor/ataxia syndrome. Ataxia can also develop as a result of stroke, multiple sclerosis, tumors, alcoholism, peripheral neuropathy, metabolic disorders, and vitamin deficiencies.

• QHow is acute disseminated encephalomyelitis (ADEM) diagnosed?

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  A Dr. Darshan N. Shah, MD , Neurology, answered on behalf of NorthShore University HealthSystem

  Prior to a diagnosis being made, a physician will suspect ADEM when a patient presents with headache, confusion, seizures, drowsiness, loss of vision, weakness, numbness, loss of bladder control and loss of coordination of the limbs preceeded by a viral infection or vaccination.  The physician will make the diagnosis of ADEM by performing blood tests and xrays to look for signs of recent infection, examining the spinal fluid to exclude other processes which can look like ADEM, and an MRI of the brain and/or spinal cord which will show inflammation of the myelin sheaths and confirm the diagnosis. 
• QWhat is arachnoiditis?

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  A Riverside Center for Neurosciences answered

  Arachnoiditis describes a pain disorder caused by the inflammation of the arachnoid, one of the membranes surrounding and protecting the nerves of the spinal cord. The arachnoid can become inflamed due to irritation from chemicals, infection from bacteria or viruses, direct injury to the spine, chronic compression of spinal nerves, or complications from spinal surgery or other invasive spinal procedures. Inflammation can sometimes lead to the formation of scar tissue and adhesions, which cause the spinal nerves to "stick" together. Arachnoiditis can interfere with the function of one or more of these nerves and cause numbness, tingling, and a characteristic stinging and burning pain in the lower back or legs. Some people with arachnoiditis will have debilitating muscle cramps, twitches, or spasms. It may also affect bladder, bowel, and sexual function. In severe cases, arachnoiditis may cause paralysis of the lower limbs.

  This answer is based on source information from The National Institute of Neurological Disorders and Strokes.

• QHow is ataxia diagnosed?

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  A NewYork-Presbyterian Hospital answered

  Many hereditary ataxias can be identified with genetic testing. Neurologists at NewYork-Presbyterian Hospital offer testing to help confirm a diagnosis or to determine if someone has inherited an ataxia gene known to affect other family members. We also offer comprehensive genetic counseling for individuals with a family history of ataxia.
  
  Neurologists at NewYork-Presbyterian Hospital's two movement disorders centers, Columbia's Center for Parkinson's Disease and Other Movement Disorders and Weill Cornell's Parkinson's Disease and Movement Disorders Institute, are expert at accurately diagnosing ataxia and helping patients and their families manage complications of the disorder.

• QHow is ataxia treated?

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  A NewYork-Presbyterian Hospital answered

  Treatment for ataxia starts with determining the underlying cause of the symptoms, and treating it if possible. If ataxia is caused by a metabolic disorder, doctors will treat that disorder with specific medications and a controlled diet. They will treat a vitamin deficiency with vitamin therapy. Other treatments for ataxia are directed toward relieving the symptoms, and may include medications currently used to treat tremor, anxiety, and gait and swallowing disorders.

• QWhat is ataxia?

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  A NewYork-Presbyterian Hospital answered

  Ataxia is a condition in which people lose control of the muscles in their arms and legs, and develop an uncoordinated gait, lack of balance, involuntary eye movements, speech problems, and an inability to maintain normal postures and perform normal movements. Depending on its cause, ataxia may be accompanied by other neurologic symptoms such as dementia, seizures, myoclonus, dystonia, and parkinsonism.