Behavioral Dysfunctions in Parkinson’s disease is a very alarming situation-Prof. Erik


Neurology Conference Proceedings-IV

Behavioral Dysfunctions in Parkinson’s
disease is a very alarming situation-Prof. Erik

All patients have different pathologies and
need different treatment strategies

KARACHI: Behavioral Dysfunctions in Parkinson’s disease is a very alarming situation. These behavioural dysfunctions are of different types. All have different pathologies and different treatment strategies. This was stated by Prof. Erik Wolters while speaking at the Neurology conference held here recently. The three main types are dopamine deficiency syndrome, dopamine dependency syndrome and dopamine dysregulated syndrome.
In Dopamine Dependency Syndrome patients have multifactorial disorders. In this the Parkinson’s disease patients take increasing quantities of Levodopa. These patients develop addiction for Levodopa drugs. One must ask these patients if they have increased the dose of the drug without medical advice or do you have difficulty in controlling Parkinson’s disease medications, do you hide or hoard your dopamine medications. It is best treated by mood stabilizers.

Prof. Daniel Dung presenting mementoes Prof. Nasrullah and other speakers
at the Neurology Conference held at Karachi recently.

Dopamine Dysregulated Syndrome: In this condition patient may lead to impulse control disorders and it is seen in 14% of Parkinson’s disease patients. About 5% of these patients are pathologically involved in gambling while 5-7% of patients mostly females are involved in compulsive shopping buying. Pathological eating disorders are seen in 4.3% of patients, compulsive social behaviours are seen in 3.5% of Parkinson’s disease patients and treatment is with antipsychotics. Some of these patients may complain of sex addiction, they complain of sexual hyperactivity. Almost 5-6% of patients suffer from depression because they do not enjoy sex. This condition can be treated with cypoteron.
Responding to the questions in the discussion, Prof Erik said that one has to ask these questions from these patients. Most often they do not volunteer this information. That is why I ask the patient and the caregivers both should come to the doctor specially while talking about sex and gambling. We have Parkinson’s disease nurse who asks these questions to the patients. In case of sleep problems, one should ask the spouse. PD nurse asks all these questions and it is time consuming. Dopamine drugs are antidepressants and they have significant actions. They do interfere with mood. They reduce depression. Each Parkinson’s disease patient suffers from some cognitive dysfunctions like dementia. In case of continuous deterioration, you may need some other drugs.

Management of Dystonias

Prof. Daniel Dung Truong from United States talked about Clinical approach and management of Dystonias. In cervical Dystonias the patient, he said, has tremor like appearances in early stage of the disease. Oromandibular dystonia affects women more than men. Focal Dystonias involve masticator muscle. It may cause jaw closing or opening, lateral deviation protrusion, retraction. Occupational dystonia is specific for a particular task. In this condition contractures are often aggravated to performance. Some patients suffer from writer’s cramps. Using the hand may lead to worsening of the condition. He also talked about sial orrhea wherein ultrasound guided injections is preferable. Other conditions he mentioned was Gustatory sweating or Frey’s syndrome.
During the discussion he referred to the use of Botox in lingual Dystonias. He was of the view and one may get good results in many cases but you just need one bad case to go home and people will start talking about you. Botox is being used in TTH, back pain, in dentistry, cosmetics and pain disorders. It has a role in migraine as well.
Dr. Tipu Sultan from Children Hospital Lahore talked about Neurodegneration in children: Diagnostic issues in developing countries. Speaking about the signs and symptoms he pointed out that in South East Asia, consanguity is very high which is responsible for high incidence of Neurodegneration. It is disorders of heterogeneous diseases which results in progressive deterioration of neurological functions. Many cases of epilepsy may turn out to be Neurodegneration. Sometimes they are labeled as cerebral palsy and sometimes they are labeled as gray matter white matter disorders. Seizures are present in both these conditions but they are more in gray matter disorders. Seizures, cognitive functions and reflexes are seen in these patients.
Speaking about its classification, he said they are inherited or acquired. He also discussed leukodystrophies in detail. It involves the brain, spinal cord, peripheral nerves. It is extremely important to diagnose these cases early. Sometimes on routine investigations, it is difficult to differentiate. He then talked about juvenile MLD. In case the patient has large size head, it could be Alexandra’s disease. Gaucher disease is diagnosed by bone marrow examination. Niemen pick disease has onset during the first year. Presence of cherry red spots helps in early diagnosis. Many of these Neurodegneration diseases, Dr. Tipu Sultan opined cannot be diagnosed because of lack of molecular genetics. In Rett syndrome onset is in the first year. Wilson’s disease is seen in most Western countries. We see these cases because of viral infections. While diagnosing these cases one must plan for investigations from routine to special tests. Diagnosis is important for genetic counseling. Management of these patients requires multidisciplinary approach. Some NGOs provide genetic counseling service. They also provide mobility aids, treatment for infections, seizure control and vaccination. Gene therapy and stem cells can be a hope for the future for the management of these patients.
Dr.Tipu Sultan further stated that genetic diagnoses are now available at Institute of Child Health in Lahore. We do not get precise diagnosis for neurodegenerative diseases. He emphasized the importance of newborn screening for a safe tomorrow. Efforts should be made to diagnose these children at birth and start immediate treatment. Treatment is costly. He suggested that we need to establish a regional molecular genetics center. Newborn screening programme should be started to avoid problems later in life. Early diagnosis and timely treatment could ensure better outcome. He concluded his presentation by stating that for preventable and treatable neurological disorders, molecular genetics are the gold standard.
On the last day of the conference the first session was chaired by Prof. Arsalan from Shifa International Hospital. Dr. Sarfaraz Ahmad from Larkana was the first speaker who talked about spectrum of Acute Flaccid Paralysis seen in a tertiary care set up. He was of the view that early diagnosis was important for good prognosis. The prevalence of GB Syndrome is about 0.6 to 1.7 per hundred thousand cases. His presentation was based on 34 male and 21 female patients. Of these fourteen patients developed facial palsy. Almost 65% of these patients needed supportive care, 11% required ventilation. After three months treatment, 50% of the patients recovered completely. GB syndrome, he said, has high mortality and one may see dramatic response to potassium. Since treatment facilities are not available, we refer these patients to other centers. These patients may require ventilation at any time. IVIG gives good results.
Dr. Asif Iqbal talked about treatment of migraine through cupping therapy. He stated that bloodletting should be human. This therapy is being used in Multiple sclerosis, SLE with good results. It is also used in pain management. His presentation was based on twenty five patients between the ages of 20-45 years. Twenty patients were in cupping therapy and five patients in the control group. For cupping we use simple suction machine for seven minutes and then draw 30-50ml blood. Then apply lotion or honey at the site. Treatment of migraine, he opined, was safe and effective with cupping therapy. During the discussion it was pointed out that even placebo treatment is effective in many cases of migraine. One needs to plan a scientific study to prove the safety and efficacy of cupping therapy with a control group in a large sample size. Almost 70% of pain is relieved by taking simple Panadol tablets. How do you measure pain, it has to be in a scientific way. One has to be very selective while using this therapy. Prof. Wasy remarked that we need some rational for such a study. Prof. Saleem Ilyas opined that we should work on integrated measures.
Brig. Saadat Ali Khan from FUMC Rawalpindi spoke on Sleep- a mysterious human behaviour. Sleep, he said, is the key to health and longevity. It also helps in healing. His presentation was based on 435 subjects who sat in Etekaf at Faisal Mosque during the month of Ramzan. These people who sat in Etakaf had longer sleep after Ramzan. Sleep deprivation could lead to serious automobile accidents and sleep deprivation is a disaster, he added.
Dr. Salman Farooq from Karachi discussed TB Meningitis. This presentation was based on a study of 404 cases. Of these 116 had motor deficit, 87 patients had seizures while 104 patients complained of stiffness of neck. Three hundred six patients had lumbar puncture for CSF studies. One hundred forty seven patents had TB meningitis with tuberculoma. Fifteen patients showed reduction in seizures, five patients developed new infarcts. Infarction alone had a high mortality. Seventy three patients (18%) died during hospitalization. Almost 50% of patients had tuberculoma. Old age and TBM with presence of infarction had high mortality. Replying to a question he said that they did not look at the use of steroids in these patients.
Dr. Safia Awan spoke about Mini Mental State Examination in one hundred patients with three hundred controls from five hospitals. Dr. Bhojo Khealani’s presentation was on National Acute Ischemic Data Bank analysis. This study was based on analysis of stroke patients at four centers. It included 874 patients with mean age of 59.7 years. About 18% were below the age of 45 years while 19% were elderly patients.
Dr. Sajjad from Larkana gave an overview of phenotypic presentation of Occulopharyngeal Muscular Dystrophy in 47 cases. All these patients were related and belonged to one village. 62% were male. Disease onset in their study was at early age. Headache was most common in their patients and they had repetitive episodes of sore throat. Dr. Abid Saleem spoke about role of Gamma Knife Radio surgery in Multimodality Management of Craniopharyngioma. Gamma Knife Surgery, he said, has a definite role in epilepsy and movement disorders. Some of their patients had 37 months follow up and they are working with good results. Treatment related neurological deficit, he pointed out, was rare in their patients. Dr. Adnan Aslam discussed frequency of depression in patients with stroke. He emphasized the importance of early screening and identification for better quality of life. Almost 50% of these stroke patients had significant disabilities. One hundred patients with age above forty years were included in this study. SAH patients as well as those whose spouse died within six months were excluded. He opined that early diagnosis and successful intervention can lead to better outcome.
Dr. Abdul Malik discussed impact of organized stroke awareness programme which included stroke screening camps, CME on stroke for GPs, Family Physicians. These camps were organized in twenty five cities. Facilities of speech therapy and occupation therapy, he said, were not available in all the cities. There is a need for life style changes in addition to medications to successfully manage these patients. A total of five hundred thirty five patients were diagnosed at these camps. Hypertension was present in more than 58% of these patients. TIAs, Diabetes Mellitus and dyslipidaemias were the other causative factors, he added.