Neuronal intranuclear inclusion disease
- Neuronal intranuclear hyaline inclusion disease
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Neuronal intranuclear inclusion disease (NIID) refers to a group of rare degenerative nerve diseases. Signs and symptoms of NIID are widespread, progressive, and usually begin in childhood. NIID causes worsening of many of the body's activities, including balance, movement, talking, eating, and cognition. Currently the cause of NIID is not known. Both sporadic and familial cases have been described.
Neuronal intranuclear inclusion disease (NIID) is a progressive disease (worsens over time) and long term prognosis is poor. Still, the course of NIID may vary from person to person. While most cases of neuronal intranuclear inclusion disease (NIID) begin in childhood or adolescence, cases of adult onset disease have been described. In general, signs and symptoms of NIID may include:
Parkinsonism - Abnormal movements, such as difficulty starting and controlling movement, stiffness of the trunk, arms, or legs, tremor, soft voice, and decrease in facial expressions.
Gastrointestinal dysfunction - For example intestinal pseudo-obstruction
Cerebellar ataxia - Unsteady gait (walking problems), lack of balance, lack of coordination, clumsy speech pattern, and repetitive or uncoordinated eye movements.
Chorea - Involuntary muscle contractions and movements
Dystonia - Involuntary muscle contractions and movements (for more information on how dystonia and chorea differ visit the We Move Web site at: http://www.wemove.org/dys/dys.html)
Nystagmus - Uncontrolled eye movements
Dementia - Symptoms may include impaired thinking that keeps one from doing normal activities (e.g., dressing, bathing, eating), loss of emotional control, personality changes, and memory loss.
Autonomic dysfunction - Some signs and symptoms include blood pressure problems, heart problems, and trouble with breathing and swallowing.
Peripheral neuropathy - Examples of early signs and symptoms include sensations of tingling, numbness, or pain. Later symptoms may involve muscle weakness, loss of muscle control, poor digestion, and some loss of bladder and bowel control.
Over the course of 10 to 20 years NIID tends to become severely disabling and the effects become life threatening. While there is currently not a cure for NIID, research into the cause and treatment of neurodegenerative disorders like NIID is ongoing.
- Josephs KA. Neuronal intranuclear inclusion disease. Neurology. 2011 Apr 19;76(16):1368-9; http://www.ncbi.nlm.nih.gov/pubmed/21411743. Accessed 8/17/2011.
- McFadden K, Hamilton RL, Insalaco SJ, Lavine L, Al-Mateen M, Wang G, Wiley CA. Neuronal intranuclear inclusion disease without polyglutamine inclusions in a child. J Neuropathol Exp Neurol. 2005 Jun;64(6):545-52; http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1402362/?tool=pubmed. Accessed 8/17/2011.