I had a strange episode the other night.
I woke up and thought I had had a stroke. My legs were like jelly, my arms had no strength, and movement was very slow motion. My brain was functioning quite well, although I had no volume to my speech.
I contemplated ringing the ambulance, but decided to stay calm , breathe deeply and do all the tests the doctors would do to diagnose a stroke. I took an asprin, and lay there slowly trying to regain control. It took 30 minutes, and slowly my strength came back. Was it a panick attack, was it a mini stroke, was it something weird? The CT scan the following morning came back clear, except for a diagnosis of, "normal scan. idiopathic calcification of the basal ganglia.'

Normal, it stated clearly. In fact, it was so normal that my doctor didn't even look at the result, until I brought it up. But when I asked her opinion about it, she didn't have a clue what it was. I have been feeling dizzy, slightly off balance, have aching muscles for 2 years, occasional facial neuropathy, headaches, and strange depression, which I never realy had. I am 52, but the doctors cannot work it out. I should be healthy.

This page will attempt to provide information about this 'rare disease", as I gather data from as many places as possible. It may not be the cause of my present symptoms, but so far in my research, it appears that it will undoubtedly have some effects at one point in my life. If anyone has any information, please add it to the page. Please click on my post headlines to take you direct to the relevant sites.Cheers.

Sunday, May 16, 2010

Wilsons Temperature Syndrome and Hypothyroidism

The articles presented on the WTS site are very interesting, to say the least. Hypothyroidism is a major cause of Calcification of the Basal Ganglia, yet finding thyroid problems seems to be subject to a lot of hit and miss diagnosis. Medicos have concluded that the results from the standard Thyroid tests, are not fully reliable, which is a remarkably important suggestion for those who have been found to have brain calcifications. If a patient has performed all the blood work ups and everything has come up "fine", it takes a dedicated team effort to try to find alternative possibilities , especially for this particular obsure disease. Due to lack of knowledge and lack of time, you doctor may not have heard of any further possible thyroid problems, and will find it easier to refer you to a neurologist or an endocronologist, for further testing.
However, ,as is explicitly explained on the Wilson Temperature Syndrome site, Hypothyroidism is still a high possibility, even though the hormone tests suggest all is well.
A simple method of testing by taking your temperature 3 times a day, for a period of time, could give you a better indication if you, in fact, do have possible thyroid misfunction, which can be corrected by following the W3 protocol. I am going to persue this angle, and will follow up, in time, with the results.
For all the information on WTS, please go to http://www.wilsonstemperaturesyndrome.com/, and decide for yourself  if this could be an approach to investigate.

Thursday, April 29, 2010

The most common ailments as per the Fahrs disease registry

Movement disorder is the most common manifestation of idiopathic basal ganglia calcification, accounting for about 55% of the symptomatic patients. Parkinsonism was seen in 57%, chorea in 19%, tremor in 8%, dystonia in 8%, athetosis in 5%, and orofacial dyskinesia in 3%. Other neurologic manifestations included cognitive impairment, cerebellar signs, speech disorder, pyramidal signs, psychiatric features, gait disorders, sensory changes, and pain.MORE
Idiopathic basal ganglia calcification
By
Shaheda N Azher and Joseph Jankovic
Last reviewed
October 1, 2009 

ATP- the fuel of life

If ATP depletion is indicated in basal ganglia calcification, new research into understanding the molecule is of great importance. Scientists are at the brink of understanding, as the latest news shows:

ScienceDaily (Mar. 9, 2010) — Researchers at the Louisiana State University Health Sciences Center have figured out how ATP is broken down in cells, providing for the first time a clear picture of the key reaction that allows cells in all living things to function and flourish

"ATP is the fuel of life. It's an energy currency molecule -- the most important source of chemical and mechanical energy in living systems," explains Sunyoung Kim, the associate professor who oversaw the research published Feb. 19 in the Journal of Biological Chemistry. MORE

Where is the Basal Ganglia?

A Professors view

Chorea and the Basal Ganglia

Chorea consists of involuntary, irregular movements that may either take on a "worm-like" appearance called athetosis or a more proximal flinging movement called ballismus
Calcium Metabolism- Hypocalcemia, most frequently in the setting of hypoparathyroidism, can cause chorea, which improves when calcium is corrected

MORE at Medscape

Dystonia and the Basal Ganglia

Dystonia is a movement disorder characterized by involuntary, sustained, patterned, and repetitive muscle contractions leading to twisting movements or abnormal postures. Dystonic movements may be classified by their anatomical distribution or by their clinical characteristics, and can be further categorized as primary (idiopathic) or secondary (symptomatic) in regard to etiology.

Calcium Metabolism. Hypocalcemia from idiopathic hypoparathyroidism may cause paroxysmal dystonia and choreoathetosis, which, rarely, may be kinesigenic. It is associated with basal ganglia calcification on neuroimaging.

MORE at Medscape.

Parkinsonism, iron overload and calcium metabolism

Calcification of the Basal Ganglia and Fahrs syndrome has been linked to a condition called "Parkinsonism", as many of the late onset symproms mimic the effects of Parkinsons disease.However, calcification is not a standard symptom of Parkinsons, and therefore, CBG is not classified under that particular disease. Getting to the bottom of WHAT causes the calcification is the primary objective of research.A decline in ATP production appears to be the most likely culprit, as is an overload of many minerals.There are many possibilities, but until there is a breakthrough, the medical world is left to study and apply their funding to the most appropriate (profitable and prolific?)disease studies.Medical breakthroughs, however are occurring in other areas, as our knowledge and technology increases.In my opinion, it is not unlikely that in the near future we will find that the majority of brain diseases, like Parkinsons, Dementia, Altzheimers, and even MS, are all interlinked.The new study on iron overload on the brain could well apply to CBG, as numerous studies have indicated metal toxicity as a possible cause.

"Disorders of calcium metabolism may occur in association with parkinsonism. Hypoparathyroidism has been reported to cause parkinsonism both in the presence and absence of basal ganglia calcifications. It may occur as a late complication after thyroidectomy[5] and may be responsive to levodopa in some cases.[6] Pseudohypoparathyroidism, characterized by end-organ resistance to normal endogenous parathyroid hormone, may be associated with parkinsonism in up to 4 to 12% of patients, either with or without evidence of basal ganglia calcifications,[7] and may respond partly to normalization of serum calcium.[8] Hyperparathyroidism due to parathyroid adenomas can rarely cause parkinsonism, which is reversible after surgical removal of the tumor.[9] Bilateral subcortical calcification involving the basal ganglia and cerebellum, often labeled as Fahr's disease, represents a heterogeneous collection of disorders that are not associated with a known disorder of calcium metabolism. Movement disorders in Fahr's disease most commonly present as parkinsonism (55%), often in association with dementia, cerebellar signs, or other hyperkinetic movements including chorea, tremor, and dystonia.[10] "
MORE from Medscape

Hypoparathyroidism and pseudohypoparathyroidism

Physiological intracranial calcification occurs in about 0.3-1.5% of cases. It is asymptomatic and detected incidentally by neuroimaging. Pathological basal ganglia calcification is due to various causes, such as: metabolic disorders, infectious and genetic diseases. Hypoparathyroidism and pseudohypoparathyroidism are the most common causes of pathological basal ganglia calcification. Besides tetany and seizures this condition is presented by parkinsonism and dementia. Such parkinsonism does not respond to drugs containing levodopa. Infections (toxoplasmosis, rubella, cytomegalovirus, cysticercosis, AIDS) give multiple and asymmetric intracranial calcification. Inherited and neurodegenerative diseases cause symmetrical, bilateral basal ganglia calcification which is not related to metabolic disorders. Since adequate treatment of hypoparathyroidism may lead to marked clinical improvement, serum concentration of calcium, phosphorus, and parathyroid hormone (PTH) is suggested to be determined in all individuals with calcification of the basal ganglia to rule out hypoparathyroidism . MORE

From the Department of Internal Medicine, KKGH, Hafr Al Batin, Saudi Arabia
Basak R. OMJ. 24, 220-222 (2009); doi:10.5001/omj.2009.43

Further report on Herpes Simplex and basal ganglia

More reports on herpes virus causing basal ganglia involvement:

"Unusual progression of herpes simplex encephalitis with basal ganglia and extensive white matter involvement'


In herpes simplex encephalitis (HSE), the gray matter of the temporal and frontal lobes is predominantly destroyed by many foci of hemorrhage and necrosis.1 The lesions usually begin either unilaterally or bilaterally in the medial temporal cortex with bilateral spread along limbic pathways to the orbital frontal lobe and insular cortex. Parietal, occipital, brainstem, internal capsule, and cingulate gyrus involvement often occurs with further spread, but the basal ganglia and lobar white matter are relatively spared.2 Here, we report a case of HSE with basal ganglia and white matter involvement evident on MRI during the acute stage, which presented with unusual and severe progression. When there was no response to medical management of HSE, we had to use surgical decompression. Pathological findings of HSE during the acute stage indicated involvement of the white matter. To our knowledge, this is the first report which shows both MR and pathological findings in the white matter during the acute stage of HSE. MORE

Department of Neurology, National Hospital Organization Okayama Medical Center, 1711-1 Tamasu Kitaku, Okayama 701-1192, Japan
E-mail: ymanabe@okayama3.hosp.go.jp

©Copyright O. Yoichi et al., 2009
Licensee PAGEPress, Italy
Neurology International 2009; 1:e9
doi:10.4081/ni.2009.e9

Could cerebral infectious diseases be a cause?

Older article regarding the condition.
Extract as follows:

Among cerebral infectious diseases a variety of conditions, including toxoplasmosis, cysticercosis, paragonimiases, trichinosis, torulosis, generalized bacterial sepsis, congenital syphilis, and infection with viruses such as herpes simplex and cytomegalo-virus present with cerebral calcifications.

(citation: K. voigt, M Sauer, and Th. Luthardt/ University Children's hospital and Neurological Clinic with department for Neurophysiology, University Freiburg i. BR., FRG) MORE

American Society of Microbiology article

This article from the american society of Microbiology is very in depth, and covers research into the Human herpesvirus 6 (HHV-6). The research suggest the possibility of HHV- being a co contributor to MS and other brain diseases. Please read the full citation for an overview.

The high fever, characteristic of HHV-6 primary infection, is often accompanied by seizures, and HHV-6 reactivation in immunocompromised patients is associated with severe encephalitis and/or encephalopathy. There have been case reports on immunocompetent and immunocompromised adults with severe CNS disease due to HHV-6 active infection, which was characterized by a fulminant multifocal demyelinating disease (32, 115, 262, 313, 329, 410). The neuroinvasiveness of the virus is proven by the fact that HHV-6 DNA is frequently detected in specimens from divergent regions of the brain (incidence rate of individuals with positive HHV-6 PCR on brain tissue, 32 to 85%) (60, 61, 83, 110, 249). Both A and B variants of HHV-6 have been found in a proportion reflecting their seroprevalence (the B variant being approximately three times more frequent than the A variant). Some patients harbor both variants, though at different locations within the brain (60). However, in a large prospective study including 2,716 children with acute ES, either HHV-6A or HHV-6B DNA, but not both, was detected in the CSF during the active phase (153). In patients with dual infection, only HHV-6A persisted in CSF, which may suggest that HHV-6A has greater neurotropism. Altogether, these studies prove that the brain is an important site for active and latent HHV-6 infection
( Clinical Microbiology Reviews, January 2005, p. 217-245, Vol. 18, No. 1 MORE


0893-8512/05/$08.00+0 doi:10.1128/CMR.18.1.217-245.2005

Copyright © 2005, American Society for Microbiology. All Rights Reserved.)

What is Fahrs syndrome?

Fahrs Syndrome
(Also Called 'Fahrs', 'Familial Idiopathic Basal Ganglia Calcification')

Synonym(s): Familial Idiopathic Basal Ganglia Calcification

What is Fahr's Syndrome?
Fahr's Syndrome is a rare, genetically dominant, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement, including the basal ganglia and the cerebral cortex. Symptoms of the disorder may include deterioration of motor function, dementia, seizures, headache, dysarthria (poorly articulated speech), spasticity (stiffness of the limbs) and spastic paralysis, eye impairments, and athetosis (involuntary, writhing movements). Fahr's Syndrome can also include symptoms characteristic of Parkinson's disease such as tremors, muscle rigidity, a mask-like facial appearance, shuffling gait, and a "pill-rolling" motion of the fingers. These symptoms generally occur later in the development of the disease. More common symptoms include dystonia (disordered muscle tone) and chorea (involuntary, rapid, jerky movements). Age of onset is typically in the 40s or 50s, although it can occur at any time in childhood or adolescence.

Is there any treatment?
There is no cure for Fahr's Syndrome, nor is there a standard course of treatment. Treatment addresses symptoms on an individual basis.

What is the prognosis?
The prognosis for any individual with Fahr's Syndrome is variable and hard to predict. There is no reliable correlation between age, extent of calcium deposits in the brain, and neurological deficit. Since the appearance of calcification is age-dependent, a CT scan could be negative in a gene carrier who is younger than the age of 55.

What research is being done?
The NINDS supports and conducts research on neurogenetic disorders such as Fahr's Syndrome. The goals of this research are to locate and understand the actions of the genes involved in this disorder. Finding these genes could lead to effective ways to treat and prevent Fahr's Syndrome. MORE

Could EB and Herpes Zoster be involved?

I read with interest on a site that the EB virus and the related Herpes virus has come under investigation , as to being a possible cause or contributor to the brain calcification process. I will expand on this, but here is just a small snippet;

Herpes zoster:
This can involve any dermatome, including the lower sacral dermatome. However, as lower sacral dermatomal zoster is much less common than genital herpes, so-called "recurrent zoster" is usually recurrent HSV infection.

Prodromal neurological symptoms of herpes zoster comprise pain rather than the typical paraesthesiae of recurrent herpes simplex. The rash of zoster is often intensely pruritic and spreads throughout the dermatome, evolving through papular, vesicular and crusting stages (Box 9). It usually lasts two to four weeks. The most troubling symptom is usually pain, which ranges from mild to severe, and from burning to lancinating (case history, Box 10). Paraesthesiae, or anaesthesia and allodynia (pain induced by touch, often from trivial stimuli), can accompany severe pain. The pain may be self-limited or persist beyond the rash for up to a year ("postherpetic neuralgia").22,25 Another relatively common complication is zoster ophthalmicus (2%–4%), which follows involvement of the first division of the fifth cranial (trigeminal) nerve. It ranges from keratitis to the more severe iritis.26

In about 1% of immunocompromised people with herpes zoster, the virus spreads to the eye, brain or liver. Prolonged pain is uncommon. People with AIDS may have recurrent or prolonged zoster or multiple dermatomal involvement, and zoster in a person at risk of HIV may be an indicator of unrecognised HIV infection.27 Herpes zoster during pregnancy is not associated with intrauterine infection.MORE

Gene Reviews-updated Sept 2007

Disease characteristics. Familial idiopathic basal ganglia calcification (FIBGC) is a neurodegenerative disorder with characteristic calcium deposits in the basal ganglia and other brain areas visualized on neuroimaging. Most affected individuals are in good health during childhood and young adulthood and typically present in the third to fifth decade with gradually progressive neuropsychiatric and movement disorders. The first manifestations often include clumsiness, fatigability, unsteady gait, slow or slurred speech, dysphagia, involuntary movements, or muscle cramping. Seizures of various types occur frequently. Neuropsychiatric symptoms, often the first or most prominent manifestations, range from mild difficulty with concentration and memory to changes in personality and/or behavior, to psychosis and dementia.

Diagnosis/testing. The diagnosis of FIBGC relies on visualization of bilateral calcification of the basal ganglia on neuroimaging; presence of progressive neurologic dysfunction; metabolic, infectious, toxic, or traumatic cause; and a family history consistent with autosomal dominant inheritance. The gene or genes responsible for FIBGC are unknown. Linkage to chromosome 14q has been established in one family.

Management. Treatment of manifestations: pharmacologic treatment to improve anxiety, depression, obsessive-compulsive behaviors, and dystonia; oxybutynin for urinary incontinence; appropriate antiepileptic drugs (AEDs) for seizures. Surveillance: annual neurologic and neuropsychiatric assessments. Agents/circumstances to avoid: cautious use of neuroleptic medication as it may exacerbate extrapyramidal symptoms. Other: generally poor response of parkinsonian features to levodopa therapy.

Genetic counseling. Familial idiopathic basal ganglia calcification is inherited in an autosomal dominant manner. The proportion of cases caused by de novo gene mutations is unknown. Offspring of an affected individual have a 50% risk of being affected. Prenatal testing is not available. MORE