Forced physical exercise can delay or slow down progression of Parkinson’s Disease in some patients - Prof. Erik

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Neurology conference Proceedings

Forced physical exercise can delay or
slow down progression of Parkinson’s
Disease in some patients - Prof. Erik

Biotinidase deficiency patients treated with Pharma grade
5-20mg of biotin per day resolves seizures and ataxia
within hours or days - Dr. Bushra Afroze

KARACHI: Our insight into Parkinson’s disease has changed a lot during the last decade. It is the most widely accepted model of behavioral disorders suggesting an activity out of balance. This was stated by Prof. Erik Wolters. Who was one of the invited guest speakers at the 21st National Neurology Conference Organized by Pakistan Society of Neurology at Karachi from March 28-30, 2014. This session was chaired by Prof. Alam Ibrahim Siddiqui from Chandka Medical College Hospital, SMBBMU Larkana.
Prof. Erik Wolters dicussed in detail about motor behavioral disorders, cognitive behavioural disorders like Attention Deficit Hyperactivity Disorders (ADHD), Mania, Obsessive Compulsive Disorders (OCD), ICD etc. Tremors, he said, are present in about 60% of patients of Parkinson’s disease.

Parkinson’s disease, he said, was a complex motor behavioural disorder. He also briefly referred to its signs and symptoms and pointed out that Dopamininetic treatment is quite effective and patients who suffer from Parkinson’s disease will benefit from it. He then also talked about progressive supranuclear palsy. Some patients might suffer form lower body half Parkinson’s and others may suffer form upper body half disorders. Some of it is compensated by reactivation movements. Parkinson’s disease is diagnosed when the first sign of Parkinson’s is present. He also briefly talked about most common non-motor symptoms of the disease like sleep disorders and sensory problems. Some strategies like forced physical exercise might delay and slow down the progression of the disease. Studies have revealed up to 35% improvement in motor functions after forced physical exercise in Parkinson’s disease patients. Physical exercise not only improves motor functions but also cognitive functions. Some patients are effectively controlled with forced physical exercise.

Stem Cells, Prof.Erik stated also offer neuro protection as well as neuro restoration by interfering in destructive pathways. However, it is a long way that we will be able to use stem cells in our patients. Surgery is very good for some patients as they will get symptomatic relief and it will improve their daily life functions. However, this treatment is accompanied by some complications which are short lived. Some of these symptoms are dopamine dependent. Treatment related complications include response fluctuations in patients on Levodopa, dyskinesia, anxiety, panic attacks, pain, fatigue, confusion. Efficacy of Levodopa, he said, is limited as it has short half life. He then talked about use of dopamine receptors and also showed video clips of some patients with motor fluctuations. It is very much bothering for the patients and also brings down their quality of life. They not only suffer from pain, restlessness but also irritability, He also referred to continuous use of Levodopa through intestinal infusion which is very expensive but through the use of this pump, we are able to give a new life to these patients. He also talked about De-brain stimulation.
Answering a question regarding the efficacy of frequent use of tablets or liquids instead of infusion pump for continuous supply of Levodopa, he said, it does improve stimulation but there is a danger that the patient might get more Levodopa and it can lead to some GI problems. Some research is going on like use of some gel or subcutaneous supply of dopamine bypassing the stomach or to have some patch but so far these experiments have not been successful. However, in the days to come we might see some positive developments in this area. It may be possible to start some patients on Levodopa treatment with patch to provide continuous stimulation. Cord blood stem cells are available and it might also be helpful as they will not cross the blood brain barriers. Efficacy of Stem Cells therapy for Parkinson’s disease patients has not yet been proved and it is not approved either by the FDA or United States or in Europe. Its success is all anecdotal, they are supplied in blood. Prolonged half life of Levodopa will be helpful but it can still stimulate motor fluctuations, Dr. Erik added.
Dr. Bushra Afroze Clinical Geneticist from Aga Khan University was the next speaker and she talked about Biotinidase deficiency- Clinical Presentation, Diagnosis and Treatment. Biotinidase deficiency, she said, is a metabolic disorder. Biotin cycle is present in food in tiny amount and in case of deficiency it is not possible to supplement it by food. Speaking about its clinical spectrum she said it has neurological, dermatological, ophthalmological and auditory problems and these patients can visit any of the above four clinics. Symptoms can be seen in newborn, children, and adults. The neurological features include epilepsy not responding to Anti Epileptic Drugs, ataxia is more common, progression encephalopathy has its own pathology. He also talked about neuro radiological features. Dermatological features include rashes, eczema not responding to drugs. Ophthalmic features include visual impairment, abnormal eye movement, corneal ulceration, audiology features like sensorineural hearing loss (SNHL). Basic biochemistry is available in diagnosis. Definite diagnosis is important, consanguinity in families is a risk factor and treatment is difficult. Untreated not responding eczema can give a clue for diagnosis. Timely diagnosis and adequate treatment is effective though some problems will be there. Treatment consists of 5-20mg of biotin per day. It is the food grade biotin which is available in the market but one needs Pharma grade biotin for treatment irrespective of age and weight. Seizures and ataxia resolves within hours or days. Coetaneous manifestations resolve within weeks. Visual abnormalities, hearing loss, developmental delay is usually irreversible even after treatment. Hence patients should be picked up before that happens. Surveillance is important as it is requires life long treatment and it must be continued. Non compliance of treatment will result n recurrence of symptoms within days or weeks while family counseling is extremely important, Dr. Bushra added.