About idiopathic basal ganglia calcification
What is idiopathic basal ganglia calcification?
Familial idiopathic basal ganglia calcification (FIBGC) is a rare neurological disorder characterized by the presence of abnormal calcium deposits (calcifications) of unknown cause. Associated symptoms include progressive deterioration of cognitive abilities (dementia), loss of contact with reality (psychosis), mood swings and loss of acquired motor skills. As the condition progresses, paralysis may develop that is associated with increased muscle stiffness (rigidity) and restricted movements (spastic paralysis). Additional abnormalities may include relatively slow, involuntary, continual writhing movements (athetosis) or chorea, a related condition characterized by irregular, rapid, jerky movements.
What are the symptoms for idiopathic basal ganglia calcification?
You may have no symptoms at all. But if you do, there are usually two types: either movement-related or psychiatric. The exact symptoms depend on which part of your brain is affected.
Movement symptoms:
- Clumsiness
- Walking unsteadily
- Talking more slowly than usual, or slurring your words
- Difficulty swallowing
- Involuntary movement of arms or legs
- Cramped muscles
- Stiff arms and legs (called spasticity)
- Tremors, muscle stiffness, lack of facial expression
Psychiatric symptoms:
- Poor concentration
- Lapses in memory
- Mood changes
- Psychosis, or being out of touch with reality
- Dementia
Other symptoms:
- • Tiredness
- • Migraines
- • Seizures
- • Vertigo, or dizziness
- • Not being able to control when you urinate (called urinary incontinence)
- • Impotence
What are the causes for idiopathic basal ganglia calcification?
Basal ganglia calcification sometimes happens when you age, but many times comes from genes passed to you by your parents. You only need one faulty gene from one parent to get the illness. Your parents may have symptoms of it, but sometimes they don’t.
Calcium build-up in your basal ganglia can also happen because of infection, problems with your parathyroid gland, and for other reasons. When it happens this way, it is also known as basal ganglia calcification, but is different from the genetic form of the disease.
What are the treatments for idiopathic basal ganglia calcification?
There is no cure, but there is treatment for the symptoms. For example, if you have seizures, certain anti-epileptic medicines can help. Or if you have migraines, you can take medicine to both prevent and treat them.
If you are anxious or depressed, see your doctor. There are medicines to help with mood issues.
If you have this condition, it’s a good idea to see your doctor or specialist every year to see if it’s changed or advanced.
What are the risk factors for idiopathic basal ganglia calcification?
Idiopathic basal ganglia calcification is a hereditary, genetic condition marked by abnormal calcium deposits in parts of the brain that regulate movement. Basal ganglia, cerebral, and cortical regions (parts of the brain) have calcium deposits that cause atrophy. Motor and mental problems result from the considerable neuron (brain cell) loss caused by this mineral deposit.
There is a paucity of molecular and genetic evidence about the molecular genetics of Fahr's syndrome. However, one of the genes responsible for idiopathic basal ganglia calcification has been identified as IBGC1. Chromosomes 8 and 2 are found to be a second locus for the genetic pathogenesis of idiopathic basal ganglia calcification. Idiopathic basal ganglia calcification typically affects people in their 40s and 50s, though it can also strike children or teenagers. Less than one in 1,00,000 people have the condition.
Diagnosis for idiopathic basal ganglia calcification
A person's overall health, family genetic history, personal medical history, and genetic history are all factors that neurologists and psychiatrists typically consider while evaluating the patient. Among the diagnostic procedures used in idiopathic basal ganglia calcification are:
Imaging
Typically, a CT scan is advised to look for calcification. Additionally, an MRI is sometimes required.
1. A blood test
Phosphorus, manganese, and alkaline phosphatase are also advised along with serum calcium.
Cerebrospinal fluid analysis (CSF analysis) is advised to look for infections and autoimmune diseases.
As many of the symptoms of idiopathic basal ganglia calcification are similar to those of other neurological and neuropsychiatric illnesses, diagnosing idiopathic basal ganglia calcification needs considerable competence. Clinical symptoms are typically considered during diagnosis, along with other potential causes being ruled out.
2. Dermatological conditions
It is associated with calcification of the basal ganglia. Lipoid proteinosis is one such illness that causes symptoms like seizures, dementia, hair loss, photosensitivity, and dwarfism.
3. Infectious conditions
This condition also participates in the calcification of the basal ganglia. Idiopathic basal ganglia calcification is linked to infections like HIV, herpes, rubella, and toxoplasmosis
Symptoms
Clumsiness,Walking unsteadily,Talking more slowly than usual, or slurring your words,Difficulty swallowing,Involuntary movement of arms or legs,Cramped muscles,Stiff arms and legs (called spasticity),Tremors, muscle stiffness, lack of facial expression
Conditions
Calcium builds up in your brain, usually in the basal ganglia, the part of your brain that helps control movement
Drugs
NA
Is there a cure/medications for idiopathic basal ganglia calcification?
Idiopathic basal ganglia calcification, a rare neurological condition marked by symmetrical and bilateral basal ganglia calcification, is caused by familial idiopathic basal ganglia calcification. Other parts of the brain, including the cerebral cortex, thalamus, and dentate nucleus, may also calcify. There are many different clinical manifestations of the illness, most of which have neuropsychiatric characteristics and mobility problems. Parkinsonism, ataxia, headache, seizures, vertigo, stroke-like episodes, orthostatic hypotension, tremor, dysarthria, and paresis are further clinical characteristics.
Symptoms of the condition are cognitive impairment, depression, hallucinations, delusions, manic symptoms, anxiety, schizophrenia-like psychosis, and personality change are psychiatric characteristics that have been documented by professionals.
Diagnosis of idiopathic basal ganglia calcification
Other disorders that might result in cerebral calcifications are included in the differential diagnosis of familial idiopathic basal ganglia calcification. The site of the calcification and the clinical picture are crucial factors in the diagnosis.
Cure or medication for idiopathic basal ganglia calcification
These are the available medication and treatments -
1. Idiopathic basal ganglia calcification patients are stabilized using a variety of therapy modalities.
2. The major objectives are to reduce symptoms and maintain the patient's comfort and pain-free state.
3. Pharmacological medications are frequently used to treat psychosis, sadness, and anxiety. To treat seizures, antiepileptic medications like carbamazepine and benzenes are used.
4. Lithium and other antipsychotic medications are used to treat depression and mood swings.
5. Lithium should be used cautiously, though, as it might exacerbate gait problems such as lurching and swaying
6. Vitamin D Alpha-hydroxy vitamin D3 dosages may reduce idiopathic basal ganglia calcification symptoms.
7. Idiopathic basal ganglia calcification requires interdisciplinary treatment from caretakers, genetic counselors, psychiatrists, neurologists, endocrinologists, and other medical professionals.
8. There are no established guidelines for managing idiopathic basal ganglia calcification at the moment. Doctors and other medical professionals treat patients on an individual basis.
Symptoms
Clumsiness,Walking unsteadily,Talking more slowly than usual, or slurring your words,Difficulty swallowing,Involuntary movement of arms or legs,Cramped muscles,Stiff arms and legs (called spasticity),Tremors, muscle stiffness, lack of facial expression
Conditions
Calcium builds up in your brain, usually in the basal ganglia, the part of your brain that helps control movement
Drugs
NA