History

Fact Explanation
History of long standing hypertension, diabetes, hyperlipidiemia [11] These are risk factors for cerebrovascular events which can cause infarction of the basal ganglia and subsequent secondary parkinsonism [11]
History of episodes of inability to talk, weakness of the body [5] [11] These features usually suggest underlying cause for secondary parkinsonism such as stroke. They may give a history of strokes and transient ischemic attacks. In vascular parkinsonism, tremor is less common. It's also known as lower body parkinsonism with rigidity. Additionally swallowing problems, urinary incontinence can occur. [5] [11]
History of long standing treatment for psychiatric disease [1] Long term treatment of anti-psychotics/ neuroleptics can cause extensive blockade of the dopamine receptors and produce extrapyramidal side effects such as parkinsonian features and these patients give a history of diseases such as schizophrenia with hallucinations, delusions, or negative symptoms [1]
Gait apraxia, urinary incontinence, memory impairment [10] [11] These are features of Normal pressure hydrocephalus which can cause secondary parkinsonism. This is due to involvement of vestibular function. Additionally gait disturbance, urinary incontinence, and dementia or cognitive decline. The gait disturbance, which is commonly the first symptom, and it's called “glued” or “magnetic” gait. Urinary incontinence due to autonomic dysfunction and memory impairment may occur in late stages of parkinsonism as well. [10] [11]
Rest tremor [1] [2] [5] This is a core symptom and it's unilateral, and disappears during activity and sleep and mainly seen in the distal part of an extremity. It is characteristically seen in the hand and is known as pill-rolling tremor. It can also be seen in the lips, chin, jaw and legs however it spares neck/head or voice. The pathophysiology is thought to be due to degeneration of a subgroup of midbrain (A8) neurons, as this area is spared in Parkinson patients not having the tremor. [1] [2]
Slowness in movements [1] [2] [5] This is a core symptom and usually seen in disorders involving basal ganglia and there are planning, initiating and executing movement are difficult and also there's a problem in performing sequential and simultaneous tasks. Fine motor activities of daily living such as buttoning, using utensils maybe intially impaired but this slowness maybe affected with emotions. Patients are seen to become excited and even do quick movements such as running in an emergency situation. This phenomenon is known as kinesia paradoxica [1] [2]
Stiffness, pain and rigidity of muscles [1] [2] [5] There's increased resistance, and it's present throughout the range of passive movement particularly in a limb. when it occurs with tremor in the hand this is known as "cogwheel rigidity". But it can be seen even in proximal muscles such as neck, shoulders, hips. Commonly shoulder stiffness and pain is one of initial features [1] [2]
Shuffling gait and postural disturbances [1] [2] Abnormal axial postures such as anterocollis, scoliosis may occur due to rigidity of axial muscles and flexed posture is usually a late feature. Gait is abnormal with having small steps and maybe occasionally with freezing [1] [2]
Freezing or periods of akinesia [1] [2] This is one of the disabling symptoms and commonly seen in men, and commonly affects the legs where there are moments of motor blocks in walking. [1] [2]
Abnormalities in speech [1] [2] Dysarthria, hypophonia and monotonous soft speech can occur due to bulbar dysfunction and thought to be due to orofacial–laryngeal bradykinesia and rigidity. Sometimes there can be word finding difficulties, known as “tip-of-the-tongue phenomenon [1] [2]
Difficulty in swallowing [1] [2] Dysphagia is due to bulbar dysfunction and could be due to inability to initiate the swallowing reflex or due to prolongation of movements of larynx oesophagus [1] [2]
Drooling of saliva [1] [2] Because of dysphagia, there will be drooling of saliva [1] [2]
Difficulty in respiration [1] [2] Muscular rigidity, cervical arthrosis or restricted muscle movement in the neck cause obstructive respiratory disease and chest wall rigidity causes , restrictive pattern. In the late stages pneumonia can occur [1] [2]
Erectile dysfunction [1] [2] [6] Autonomic dysfunction also occurs and this results in erectile dysfunction [1] [2] [6]
Disturbances in sweating [1] [2] [6] Autonomic dysfunction also occurs and this results in sweating dysfunction [1] [2] [6]
Psychiatric disorders [1] [2] Depression, apathy, anxiety, hallucinations, obsessive–compulsive and impulsive behaviour are commonly seen. Exact mechanism is not fully understood. This is thought to be due to dopamine dysregulation syndrome due to use of dopaminergic drugs such as dopamine agonists [1] [2]
Sleep disturbances [1] [2] Excessive sleepiness is seen and thought to be due to 50% loss of hypocretin (orexin) neurons and maybe attributed to drugs used in treatment as well. A pre-parkinsonian state is recognized with insomnia, increase in violent dreams, talking, yelling in sleep. [1] [2]
Olfactory dysfunction, Oral pain, akathisia, genital pain [2] These are pre-parkinsonism features and thought to be due to neuronal loss in the corticomedial amygdala or to decreased dopaminergic neurons in the olfactory bulb. [2]
Disturbances in vision [8] This maybe due to slow visual processing speed leading to decline in visual perception. Alternatively some drugs used in the treatment can also have adverse ocular reactions [8]
References
  1. FERNANDEZ H. H.. Updates in the medical management of Parkinson disease. Cleveland Clinic Journal of Medicine [online] December, 79(1):28-35 [viewed 17 September 2014] Available from: doi:10.3949/ccjm.78gr.11005
  2. JANKOVIC J. Parkinson's disease: clinical features and diagnosis. Journal of Neurology, Neurosurgery & Psychiatry [online] 2008 April, 79(4):368-376 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.2007.131045
  3. MüLLER THOMAS. Drug therapy in patients with Parkinson’s disease. Array [online] 2012 December [viewed 17 September 2014] Available from: doi:10.1186/2047-9158-1-10
  4. FRANK C, PARI G, ROSSITER JP. Approach to diagnosis of Parkinson disease Can Fam Physician [online] 2006 Jul 10, 52(7):862-868 [viewed 17 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1781092
  5. HANDLEY A., MEDCALF P., HELLIER K., DUTTA D.. Movement disorders after stroke. Age and Ageing [online] 2008 October, 38(3):260-266 [viewed 17 September 2014] Available from: doi:10.1093/ageing/afp020
  6. MATHIAS C. J. Autonomic diseases: clinical features and laboratory evaluation. Journal of Neurology, Neurosurgery & Psychiatry [online] 2003 September, 74(90003):31iii-41 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.74.suppl_3.iii31
  7. VAN DEN BERGE SIMONE A., KEVENAAR JOSTA T., SLUIJS JACQUELINE A., HOL ELLY M.. Dementia in Parkinson's Disease Correlates with α-Synuclein Pathology but Not with Cortical Astrogliosis. Parkinson's Disease [online] 2012 December, 2012:1-13 [viewed 17 September 2014] Available from: doi:10.1155/2012/420957
  8. ARMSTRONG R. A.. Visual Symptoms in Parkinson's Disease. Parkinson's Disease [online] 2011 December, 2011:1-9 [viewed 17 September 2014] Available from: doi:10.4061/2011/908306
  9. GROSSET K A. Prescribed drugs and neurological complications. Journal of Neurology, Neurosurgery & Psychiatry [online] 2004 September, 75(suppl_3):iii2-iii8 [viewed 20 September 2014] Available from: doi:10.1136/jnnp.2004.045757
  10. CONN H. O.. Normal pressure hydrocephalus (NPH): more about NPH by a physician who is the patient. Clinical Medicine [online] 2011 April, 11(2):162-165 [viewed 20 September 2014] Available from: doi:10.7861/clinmedicine.11-2-162
  11. TATSCH K., POEPPERL G.. Nigrostriatal Dopamine Terminal Imaging with Dopamine Transporter SPECT: An Update. Journal of Nuclear Medicine [online] December, 54(8):1331-1338 [viewed 20 September 2014] Available from: doi:10.2967/jnumed.112.105379
  12. BEHARI MADHURI, GUPTA PRAVEEN. Akinetic rigid syndrome: An overview. Ann Indian Acad Neurol [online] 2007 December [viewed 20 September 2014] Available from: doi:10.4103/0972-2327.31481

Examination

Fact Explanation
Elevated blood pressure [10] Hypertension is a risk factor for cerebrovascular accidents. But later in the disease postural hypotension can occur due to autonomic involvement. This is a fall in systolic blood pressure of at least 20 mmHg and diastolic blood pressure of at least 10 mmHg on standing or head-up tilt and is due to autonomic dysfunction in parkinsonism [2] [6] [7]
Aphasia, weakness of the face [10] These are features which occur as a result of cerebrovascular accident (CVA). Similarly CVA s can occur in basal ganglia and cause secondary parkinsonism as well [8] [10]
Spasticity of limbs with hyper-reflexia and extensor planter response [10] These are upper motor neuron features of lower limbs which occur as a result of cerebrovascular accident (CVA). Similarly CVA s can occur in basal ganglia and cause secondary parkinsonism as well [8] [10]
Rhythmic involuntary movements of tongue, face, and jaw, tics and torticollis [9] These are features of tardive dyskinesia due to long term treatment with dopamine receptor blocking drugs similar to parkinsonian features. [9]
neck retraction, tongue protrusion, trismus, and oculogyric crisis [9] These are features of acute dystonic reactions which occur due to dopamine receptor blocking drugs similar to parkinsonian features. Mainly seen in young adults. [9]
Gait apraxia [11] These are features of Normal pressure hydrocephalus which can cause secondary parkinsonism. This is due to involvement of vestibular function. The gait disturbance, which is commonly the first symptom, and it's called “glued” or “magnetic” gait. Gait is abnormal in Parkinson's disease with having small steps and loss of arm swing. Walking is difficult to be initiated and occasionally there are episodes of freezing [1] [2][11]
Loss of facial expression with reduced blinking rate [3] [5] Bradykinesia of the face causes loss of facial expressions [3] [5]
Resting tremor [1] [2] This is a core symptom and it's unilateral, and disappears during activity and sleep and mainly seen in the distal part of an extremity. It is characteristically seen in the hand and is known as pill-rolling tremor. It can also be seen in the lips, chin, jaw and legs however it spares neck/head or voice. The pathophysiology is thought to be due to degeneration of a subgroup of midbrain (A8) neurons, as this area is spared in Parkinson patients not having the tremor. [1] [2]
Bradykinesia [1] [2] This is a core symptom and usually seen in disorders involving basal ganglia and there are planning, initiating and executing movement are difficult and also there's a problem in performing sequential and simultaneous tasks. Fine motor activities of daily living such as buttoning, using utensils maybe initially impaired but this slowness maybe affected with emotions. Patients are seen to become excited and even do quick movements such as running in an emergency situation. This phenomenon is known as kinesia [1] [2]
Rigidity of muscles with increased resistance to passive movements [1] [2] There's increased resistance mainly objective, and it's present throughout the range of passive movement particularly in a limb. when it occurs with tremor in the hand this is known as "cogwheel rigidity". But it can be seen even in proximal muscles such as neck, shoulders, hips. Commonly shoulder stiffness and pain is one of initial features [1] [2]
Postural and limb deformities [2] Abnormal axial postures such as anterocollis, scoliosis may occur due to rigidity of axial muscles and flexed posture is usually a late feature. Striatal limb deformities can develop in some also dropped head/ bent spine due to extreme neck flexion, extreme flexion of thoracolumbar spine (camptocormia). Sometimes Pisa syndrome due to tilting of the trunk when sitting or standing can occur. [2]
Glabellar tap [3] [4] In a normal person, light tapping over the glabella produces a reflex blinking of both eyes but in patients with Parkinsonism, eyes continue to blink with tapping. This is not specific for Parkinsonism as it could also result from diffuse (frontal lobe) damage. [3] [4]
Palmomental reflex [2] [4] This is a primitive reflex and even though this is not sensitive it's more more specific than the glabellar reflex. [2] [4]
Mirror movements [2] these are unintentional movements accompany voluntary activity in homologous opposite muscles and mainly seen in early asymmetric Parkinsonism.[2]
Monotonous, soft speech [1] [2] Dysarthria, hypophonia and monotonous soft speech can occur due to bulbar dysfunction and thought to be due to orofacial–laryngeal bradykinesia and rigidity. Sometimes there can be word finding difficulties, known as “tip-of-the-tongue phenomenon. Sometimes drooling of saliva may be noted in examination due to dysphagia [1] [2]
Difficulty in breathing [1] [2] Muscular rigidity, cervical arthrosis or restricted muscle movement in the neck cause obstructive respiratory disease and chest wall rigidity causes , restrictive pattern. In the late stages pneumonia can occur [1] [2]
Reduced visual acuity, Defects in color vision [5] This is thought to be due to lack of dopamine in the retina, abnormal eye movements, or poor blinking. Color vision is blurred. [5]
Defects in saccadic and Smooth Pursuit Eye Movements [5] This is attributed to dopamine deficiency in the basal ganglia [5]
Nystagmus [5] Limitation of eye movement can cause nystagmus. [5]
Large pupils and unequal pupils [5] Large pupil with anisocoria to light adaptation is noted and thought to be due to autonomic imbalance in parasympathetic system. [5]
References
  1. FERNANDEZ H. H.. Updates in the medical management of Parkinson disease. Cleveland Clinic Journal of Medicine [online] December, 79(1):28-35 [viewed 17 September 2014] Available from: doi:10.3949/ccjm.78gr.11005
  2. JANKOVIC J. Parkinson's disease: clinical features and diagnosis. Journal of Neurology, Neurosurgery & Psychiatry [online] 2008 April, 79(4):368-376 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.2007.131045
  3. DAVIE C. A.. A review of Parkinson's disease. British Medical Bulletin [online] 2008 February, 86(1):109-127 [viewed 17 September 2014] Available from: doi:10.1093/bmb/ldn013
  4. SCHOTT J M. The grasp and other primitive reflexes. [online] 2003 May, 74(5):558-560 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.74.5.558
  5. ARMSTRONG R. A.. Visual Symptoms in Parkinson's Disease. Parkinson's Disease [online] 2011 December, 2011:1-9 [viewed 17 September 2014] Available from: doi:10.4061/2011/908306
  6. FERESHTEHNEJAD SEYED-MOHAMMAD, LöKK JOHAN. Orthostatic Hypotension in Patients with Parkinson’s Disease and Atypical Parkinsonism. Parkinson's Disease [online] 2014 December, 2014:1-10 [viewed 17 September 2014] Available from: doi:10.1155/2014/475854
  7. MATHIAS C. J. Autonomic diseases: clinical features and laboratory evaluation. Journal of Neurology, Neurosurgery & Psychiatry [online] 2003 September, 74(90003):31iii-41 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.74.suppl_3.iii31
  8. HANDLEY A., MEDCALF P., HELLIER K., DUTTA D.. Movement disorders after stroke. Age and Ageing [online] 2008 October, 38(3):260-266 [viewed 17 September 2014] Available from: doi:10.1093/ageing/afp020
  9. GROSSET K A. Prescribed drugs and neurological complications. Journal of Neurology, Neurosurgery & Psychiatry [online] 2004 September, 75(suppl_3):iii2-iii8 [viewed 20 September 2014] Available from: doi:10.1136/jnnp.2004.045757
  10. TATSCH K., POEPPERL G.. Nigrostriatal Dopamine Terminal Imaging with Dopamine Transporter SPECT: An Update. Journal of Nuclear Medicine [online] December, 54(8):1331-1338 [viewed 20 September 2014] Available from: doi:10.2967/jnumed.112.105379
  11. CONN H. O.. Normal pressure hydrocephalus (NPH): more about NPH by a physician who is the patient. Clinical Medicine [online] 2011 April, 11(2):162-165 [viewed 20 September 2014] Available from: doi:10.7861/clinmedicine.11-2-162

Differential Diagnoses

Fact Explanation
Primary (idiopathic) parkinsonism [1] [2] This is the most common cause for Parkinsonism. The diagnosis of Parkinson disease is mainly clinical with cardinal features of bradykinesia, tremor, rigidity and postural instability. There's good response with dopaminergic treatment with levodopa [1] [2]
Parkinsonism plus syndromes [1] [4] These are Progressive supranuclear palsy, Multiple system atrophy, Corticobasal degeneration and lewy body dementia. These have additional features in addition to the classic features of idiopathic Parkinson disease. Early onset of postural instability, prominent axial rigidity, early dementia, supranuclear palsy are characteristic and there's usually a poor response to treatment with Levodopa [1] [4]
Wilson disease [1] When the patient is younger that 50 years, Wilson disease need to be considered in the differential diagnosis, This is an autosomal recessive disorder associated with excess copper accumulation, Serum ceruloplasmin, 24-hour urinary copper excretion, and slit-lamp examination for Kaiser-Fleischer rings should be done [1]
Brain tumors [1] When the patient is young a structural lesion in the brain need to be considered [1]
Benign essential tremor [3] This is seen in 4% of adults over 40 years of age and this is often familial tremor with a family history of tremor. [3]
Cerebrovascular disease [1] A stoke can cause parkinsonian features when it involves basal ganglia and other additional features depending on the area of the brain involved. Dementia can also be seen (Vascular dementia) [1] [5] [6]
Alzheimer's disease [5] [6] This is the most common form of irreversible dementia affecting the elderly in USA and Europe. [5] [6]
Dementia With Lewy Bodies [7] This is the second most common cause of degenerative dementia in older people accounting for 10-15% and this also includes Parkinson's disease and primary autonomic failure. [7]
Huntington Disease [8] [9] This is an autosomal dominant disease which has a prevalence of 5-10 per 100,000 in the Caucasian population. Motor, cognitive and psychiatric disturbances are seen. [8] [9]
Chorea in Adults [10] This is a slowly progressive, generalized chorea in elderly people without mental deterioration. Underlying cause is not known [10]
References
  1. FERNANDEZ H. H.. Updates in the medical management of Parkinson disease. Cleveland Clinic Journal of Medicine [online] December, 79(1):28-35 [viewed 17 September 2014] Available from: doi:10.3949/ccjm.78gr.11005
  2. JANKOVIC J. Parkinson's disease: clinical features and diagnosis. Journal of Neurology, Neurosurgery & Psychiatry [online] 2008 April, 79(4):368-376 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.2007.131045
  3. ABBOUD H., AHMED A., FERNANDEZ H. H.. Essential tremor: Choosing the right management plan for your patient. Cleveland Clinic Journal of Medicine [online] December, 78(12):821-828 [viewed 17 September 2014] Available from: doi:10.3949/ccjm.78a.10178
  4. BURN DJ, SAWLE GV, BROOKS DJ. Differential diagnosis of Parkinson's disease, multiple system atrophy, and Steele-Richardson-Olszewski syndrome: discriminant analysis of striatal 18F-dopa PET data. J Neurol Neurosurg Psychiatry [online] 1994 Mar, 57(3):278-284 [viewed 17 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1072814
  5. GORELICK P. B.. Risk Factors for Vascular Dementia and Alzheimer Disease. Stroke [online] 2004 September, 35(11_suppl_1):2620-2622 [viewed 17 September 2014] Available from: doi:10.1161/01.STR.0000143318.70292.47
  6. CHERTKOW HOWARD, FELDMAN HOWARD H, JACOVA CLAUDIA, MASSOUD FADI. Definitions of dementia and predementia states in Alzheimer's disease and vascular cognitive impairment: consensus from the Canadian conference on diagnosis of dementia. Alzheimer's Research & Therapy [online] 2013 December [viewed 17 September 2014] Available from: doi:10.1186/alzrt198
  7. MCKEITH I. Dementia with Lewy bodies Dialogues Clin Neurosci [online] 2004 Sep, 6(3):333-341 [viewed 17 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3181810
  8. NAYAK ANNAPURNA, ANSAR RAFIA, VERMA SUNIL K., BONIFATI DOMENICO MARCO, KISHORE UDAY. Huntington's Disease: An Immune Perspective. Neurology Research International [online] 2011 December, 2011:1-7 [viewed 17 September 2014] Available from: doi:10.1155/2011/563784
  9. ROOS RAYMUND A.C.. Huntington's disease: a clinical review. Array [online] 2010 December [viewed 17 September 2014] Available from: doi:10.1186/1750-1172-5-40
  10. GRIMES D A. Late adult onset chorea with typical pathology of Hallervorden-Spatz syndrome. [online] 2000 September, 69(3):392-395 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.69.3.392

Investigations - for Diagnosis

Fact Explanation
Serum cholesterol [2] It's important to evaluate for serum cholesterol level as atherosclerosis can cause cerebrovascular disease and consequent secondary parkinsonism [2]
Fasting blood sugar [1] It's important to evaluate for blood sugar level as diabetes can increase the risk for cerebrovascular disease and consequent secondary parkinsonism [1]
Serum cerulopasmin, Serum copper and 24 hour urinary copper [3] In younger patients Wilson's disease needs to be excluded. [3]
Lumbar puncture [6] To exclude normal pressure hydrocephalus as the removal of fluid improves symptoms. It has a traid of ataxia, dementia and urinary incontinence and is recognized as a cause for secondary parkinsonism. This is known as tap test [6]
Transcranial ultrasound [12] This is important to exclude secondary parkinsonism [12]
MRI brain [3] [5] To exclude structural brain lesions such as cerebral tumors, cerebral hemorrhages, infarctions, hydrocephalus [3] [5]
Positron emission tomography (PET) and single-photon emission computed tomography (SPECT) scanning of the brain [4] [5] This gives an idea of remaining dopaminergic neurons. In PET imaging, there's an increase in F-dopa (fluorodopa) uptake in the contralateral putamen in patient's with Parkinsonism. IPT, TRODAT-1, and FP-CIT tagged with either iodine-123 or technetium-99 are used in the SPECT scanning which is based on the same principle. [4] [5]
Biopsy and histology [7] This shows degeneration of pigmented dopaminergic neurons in the basal ganglia. [7]
Slit lamp examination [3] This is done to exclude Wilson's disease in which there will be Kayser-Fleisher rings. [3]
Electrophysiological studies [8] [9] A surface electromyelogram (EMG) recording can help to detect the frequency and amplitude of a tremor, Multiple system Atrophy patients may have degeneration of Onuf's nucleus which can be detected as polyphasic potentials with a prolonged latency on urethral or anal sphincter EMG [8] [9]
Eye movement recordings [11] These can point towards hypometric saccades, supranuclear gaze problems, square wave jerks in atypical parkinsonism [11]
Valsalva manoeuvre [10] To identify any autonomic dysfunction associated with Parkinsonism [10]
References
  1. SUNG J., SONG Y.-M., EBRAHIM S., LAWLOR D. A.. Fasting Blood Glucose and the Risk of Stroke and Myocardial Infarction. Circulation [online] December, 119(6):812-819 [viewed 17 September 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.776989
  2. PIECHOWSKI-JOZWIAK B., BOGOUSSLAVSKY J.. Cholesterol as a Risk Factor for Stroke: The Fugitive?. Stroke [online] 2004 May, 35(6):1523-1524 [viewed 17 September 2014] Available from: doi:10.1161/01.STR.0000130460.09807.ec
  3. FERNANDEZ H. H.. Updates in the medical management of Parkinson disease. Cleveland Clinic Journal of Medicine [online] December, 79(1):28-35 [viewed 17 September 2014] Available from: doi:10.3949/ccjm.78gr.11005
  4. WANG LING, ZHANG QI, LI HUANBIN, ZHANG HONG. SPECT Molecular Imaging in Parkinson's Disease. Journal of Biomedicine and Biotechnology [online] 2012 December, 2012:1-11 [viewed 17 September 2014] Available from: doi:10.1155/2012/412486
  5. BROOKS D. J.. Imaging Approaches to Parkinson Disease. Journal of Nuclear Medicine [online] December, 51(4):596-609 [viewed 17 September 2014] Available from: doi:10.2967/jnumed.108.059998
  6. WIKKELSO C, ANDERSSON H, BLOMSTRAND C, LINDQVIST G. The clinical effect of lumbar puncture in normal pressure hydrocephalus.. Journal of Neurology, Neurosurgery & Psychiatry [online] 1982 January, 45(1):64-69 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.45.1.64
  7. FRANK C, PARI G, ROSSITER JP. Approach to diagnosis of Parkinson disease Can Fam Physician [online] 2006 Jul 10, 52(7):862-868 [viewed 17 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1781092
  8. GILAD R. Quantitative anal sphincter EMG in multisystem atrophy and 100 controls. [online] 2001 November, 71(5):596-599 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.71.5.596
  9. YAMAMOTO T. When is Onuf's nucleus involved in multiple system atrophy? A sphincter electromyography study. Journal of Neurology, Neurosurgery & Psychiatry [online] 2005 December, 76(12):1645-1648 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.2004.061036
  10. FERESHTEHNEJAD SEYED-MOHAMMAD, LöKK JOHAN. Orthostatic Hypotension in Patients with Parkinson’s Disease and Atypical Parkinsonism. Parkinson's Disease [online] 2014 December, 2014:1-10 [viewed 17 September 2014] Available from: doi:10.1155/2014/475854
  11. PINKHARDT ELMAR H, JüRGENS REINHART, LULé DOROTHéE, HEIMRATH JOHANNA, LUDOLPH ALBERT C, BECKER WOLFGANG, KASSUBEK JAN. Eye movement impairments in Parkinson's disease: possible role of extradopaminergic mechanisms. Array [online] 2012 December [viewed 17 September 2014] Available from: doi:10.1186/1471-2377-12-5
  12. LAUčKAITė KRISTINA, RASTENYTė DAIVA, ŠURKIENė DANGUOLė, VAITKUS ANTANAS, SAKALAUSKAS ANDRIUS, LUKOšEVIčIUS ARūNAS, GLEIZNIENė RYMANTė. Specificity of transcranial sonography in parkinson spectrum disorders in comparison to degenerative cognitive syndromes. Array [online] 2012 December [viewed 17 September 2014] Available from: doi:10.1186/1471-2377-12-12

Investigations - Fitness for Management

Fact Explanation
Full blood count [1] [2] To detect and correct any anemia prior to surgery [1] [2]
Coagulation studies [1] [2] To detect and correct any coagulopathy before surgical management [1] [2]
Renal function tests- Serum creatinine, Blood urea nitrogen [1] [2] To determine any renal dysfunction prior to anesthesia [1] [2]
Fasting blood sugar [3] It's important to evaluate for blood sugar level as diabetes can increase the risk for cerebrovascular disease and consequent secondary parkinsonism. This is important before anesthesia [3]
MRI brain [4] To exclude secondary causes for Parkinsonism prior to surgery [4]
References
  1. KUMAR A, SRIVASTAVA U. Role of routine laboratory investigations in preoperative evaluation J Anaesthesiol Clin Pharmacol [online] 2011, 27(2):174-179 [viewed 17 September 2014] Available from: doi:10.4103/0970-9185.81824
  2. SHULMAN M. A., THOMPSON B. R.. I. Not fit for a haircut ... how should we assess fitness and stratify risk for surgery?. British Journal of Anaesthesia [online] December, 112(6):955-957 [viewed 17 September 2014] Available from: doi:10.1093/bja/aeu003
  3. DAGOGO-JACK S., ALBERTI K. G. M.M.. Management of Diabetes Mellitus in Surgical Patients. Diabetes Spectrum [online] 2002 January, 15(1):44-48 [viewed 17 September 2014] Available from: doi:10.2337/diaspect.15.1.44
  4. MUQIT M M K. "Hot cross bun" sign in a patient with parkinsonism secondary to presumed vasculitis. [online] 2001 October, 71(4):565-566 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.71.4.565

Investigations - Followup

Fact Explanation
Serum cholesterol [2] It's important to evaluate for serum cholesterol level in the follow up as atherosclerosis is a risk factor for cerebrovascular disease and to prevent further events [2]
Fasting blood sugar [1] It's important to evaluate for blood sugar level as diabetes can increase the risk for cerebrovascular disease and to prevent further events [1]
References
  1. SUNG J., SONG Y.-M., EBRAHIM S., LAWLOR D. A.. Fasting Blood Glucose and the Risk of Stroke and Myocardial Infarction. Circulation [online] December, 119(6):812-819 [viewed 17 September 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.776989
  2. PIECHOWSKI-JOZWIAK B., BOGOUSSLAVSKY J.. Cholesterol as a Risk Factor for Stroke: The Fugitive?. Stroke [online] 2004 May, 35(6):1523-1524 [viewed 17 September 2014] Available from: doi:10.1161/01.STR.0000130460.09807.ec

Investigations - Screening/Staging

Fact Explanation
olfactory testing [1] This is easy and quick test. Impairment of olfaction is an early feature of Parkinson's disease and these deficits may precede clinical motor symptoms by years. therefore this can be used to diagnose asymptomatic individuals who have a high risk of developing parkinsonism [1]
References
  1. HAEHNER ANTJE, HUMMEL THOMAS, REICHMANN HEINZ. Olfactory Loss in Parkinson's Disease. Parkinson's Disease [online] 2011 December, 2011:1-6 [viewed 17 September 2014] Available from: doi:10.4061/2011/450939

Management - General Measures

Fact Explanation
Patient education [1] This is a disease which is not curative and only symptomatic improvement is done. Therefore patient education and family education is vital. they should be educated on the etiology, nature, course, prognosis, available treatment options, and importance of follow up. The residencies may need to be modified according to patient's needs, and the dementia which occurs later in the disease needs to addressed. Patients should be educated on that and necessary actions for financial issue, making the last will should be done. Support groups are present for the patient and the family. Caregiver's support is also important. [1]
Physiotherapy [2] This is important for improving gait, posture, initiation of movement. walking sticks, wheel chairs maybe needed in the late stages. This is important to prevent falls [2]
Occupational therapy [3] This is very important to improve the functional status to carry out activities of daily living. [3]
Speech therapy [4] [5] Speech problems are common in Parkinsonism. To increase vocal intensity a program called Lee Silverman Voice Treatment (LSVT) is used. It improves phonation as well as respiration [4] [5]
Diet and nutrition [6] A good nutritional support is essential. As the disease is complicated by dysphagia in late stages percutaneous endoscopic gastostomy feeding may be necessary. [6]
bladder, bowel care [6] Bladder care are important as these patients show incontinence. Anticholinergics may be useful. Self intermittent catheterization, for urinary incontinence, diapers may be needed in late stages. Constipation can occur due to drugs used in the treatment, therefore increasing fibre intake, laxatives may be needed. [6]
Skin care [7] As patients become bed ridden later, it's very important to prevent bed sores. Rolling the patient every 2 hours, air mattresses are necessary. [7]
Treatment for psychiatric disorders [6] These patients are likely to develop various psychiatric disorders, therefore anti depressants for depression may be needed. Reduction of the dosage of anti parkinson's drugs maybe necessary to reduce dementia. anxiety, sleep disorders, behavioral symptoms [6]
Treatment for orthostatic hypotension [6] Education of patients and caregivers is important to avoid rapid changes of position or straining during micturition or defecation, which can trigger orthostatic hypotension Fluid intake should be adequate with salt to ensure adequate hydration. Antihypertensive therapy may need to be discontinued. Fludrocortison, and vasoactive agents, like midodrine may be necessary as well. [6]
References
  1. KLEINER-FISMAN GALIT, GRYFE PEARL, NAGLIE GARY. A Patient-Based Needs Assessment for Living Well with Parkinson Disease: Implementation via Nominal Group Technique. Parkinson's Disease [online] 2013 December, 2013:1-6 [viewed 17 September 2014] Available from: doi:10.1155/2013/974964
  2. MORRIS ME, MARTIN CL, SCHENKMAN ML. Striding Out With Parkinson Disease: Evidence-Based Physical Therapy for Gait Disorders Phys Ther [online] 2010 Feb, 90(2):280-288 [viewed 17 September 2014] Available from: doi:10.2522/ptj.20090091
  3. STURKENBOOM INGRID HWM, GRAFF MAUD J, BORM GEORGE F, ADANG EDDY MM, NIJHUIS-VAN DER SANDEN MARIA WG, BLOEM BASTIAAN R, MUNNEKE MARTEN. Effectiveness of occupational therapy in Parkinson’s disease: study protocol for a randomized controlled trial. Array [online] 2013 December [viewed 17 September 2014] Available from: doi:10.1186/1745-6215-14-34
  4. FOX CYNTHIA, EBERSBACH GEORG, RAMIG LORRAINE, SAPIR SHIMON. LSVT LOUD and LSVT BIG: Behavioral Treatment Programs for Speech and Body Movement in Parkinson Disease. Parkinson's Disease [online] 2012 December, 2012:1-12 [viewed 17 September 2014] Available from: doi:10.1155/2012/391946
  5. RAMIG L O. Intensive voice treatment (LSVT(R)) for patients with Parkinson's disease: a 2 year follow up. [online] 2001 October, 71(4):493-498 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.71.4.493
  6. VARANESE SARA, BIRNBAUM ZOE, ROSSI ROGER, DI ROCCO ALESSANDRO. Treatment of Advanced Parkinson's Disease. Parkinson's Disease [online] 2010 December, 2010:1-9 [viewed 17 September 2014] Available from: doi:10.4061/2010/480260
  7. LOKK JOHAN, DELBARI AHMAD. Clinical aspects of palliative care in advanced Parkinson’s disease. Array [online] 2012 December [viewed 17 September 2014] Available from: doi:10.1186/1472-684X-11-20

Management - Specific Treatments

Fact Explanation
Discontinuation of antipsychotics [6] If it's drug induced parkinsonism, neuroleptics/ Antipsychotics may need to be discontinued or dose may need to be reduced [6]
Neurosurgery [2] [13] If there's a cerebral tumor in the basal ganglia region which causes parkinsonism, these needs surgical removal if possible. Also in Parkinson's disease, Hyperactivity of subthalamic neurons are noted. Therefore surgical procedures such as thalamotomy, Subthalamotomy, Pallidotomy may be helpful in advanced disease. [2] [13]
CSF shunting may be needed in normal pressure hydrocephalus. [4] CSF shunting may be needed in normal pressure hydrocephalus. [4]
Treatment for cerebrovascular disease involving basal ganglia [5] Treatment for the risk factors for atherosclerotic diseases such as anti-platelet agents, statins and anti-hypertensives are needed to prevent further episodes [5]
Treatment for encephalitis [8] [10] Viral encephalitis can cause parkinsonian features. Especially HIV, japanese encephalitis. These needs supportive management as for any encephalitis and appropriate therapy if available. [8] [10]
Treatment for meningitis [8] [9] [11] [12] Cryptococcal meningitis, tubercular and pyogenic meningitis can cause parkinsonian features. Therefore antibiotic treatment, antifungals, anti- TB therapy will be needed. [8] [9] [11] [12]
Pharmacological therapy with Dopamine replacement [6] Levodopa combined with Peripheral dopa De carboxylase inhibitor, is the treatment for early disease. Dopamine agonists are also used in early disease and thought to be having few adverse effects such as motor fluctuations. MAO-B inhibitors, such as selegiline and rasagiline, catechol-O-methyltransferase (COMT) inhibitors such as Entacapone are the other drugs which are used. Selegiline, Rasagiline may have neuroprotective effect as well. Amanatadine is an anti-viral drug that is used. These drugs can be used in combination. However the response to drugs is poor when compared with idiopathic parkinsonism. [6]
Pharmacological therapy with anticholinergics [6] These are used for the treatment of tremor. Older people are more prone to adverse effects, therefore should be used with caution. [6]
Management of dyskinesia [7] Lower doses of levodopa can be given. For wearing-off, a dopamine agonist or MAO-B inhibitor can be added. Amantadine which is a (NMDA) receptor antagonist is also effective against dyskinesias. [7]
Deep brain stimulation [1] It's a type of stereotactic surgery. Chronic high frequency electrical stimulation it is used to achieve a functional inhibition of the subthalamic neurons. This is effective in advanced Parkinson's disease. [1]
References
  1. RIZZONE M. Deep brain stimulation of the subthalamic nucleus in Parkinson's disease: effects of variation in stimulation parameters. [online] 2001 August, 71(2):215-219 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.71.2.215
  2. PATEL N. K., HEYWOOD P., O'SULLIVAN K., MCCARTER R., LOVE S., GILL S. S.. Unilateral subthalamotomy in the treatment of Parkinson's disease. Brain [online] 2003 May, 126(5):1136-1145 [viewed 17 September 2014] Available from: doi:10.1093/brain/awg111
  3. SHPRECHER D, SCHWALB J, KURLAN R. Normal Pressure Hydrocephalus: Diagnosis and Treatment Curr Neurol Neurosci Rep [online] 2008 Sep, 8(5):371-376 [viewed 17 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2674287
  4. HANDLEY A., MEDCALF P., HELLIER K., DUTTA D.. Movement disorders after stroke. Age and Ageing [online] 2008 October, 38(3):260-266 [viewed 17 September 2014] Available from: doi:10.1093/ageing/afp020
  5. SALTZ BL, ROBINSON DG, WOERNER MG. Recognizing and Managing Antipsychotic Drug Treatment Side Effects in the Elderly Prim Care Companion J Clin Psychiatry [online] 2004, 6(suppl 2):14-19 [viewed 17 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC487007
  6. MüLLER THOMAS. Drug therapy in patients with Parkinson’s disease. Array [online] 2012 December [viewed 17 September 2014] Available from: doi:10.1186/2047-9158-1-10
  7. FERNANDEZ H. H.. Updates in the medical management of Parkinson disease. Cleveland Clinic Journal of Medicine [online] December, 79(1):28-35 [viewed 17 September 2014] Available from: doi:10.3949/ccjm.78gr.11005
  8. BEHARI MADHURI, GUPTA PRAVEEN. Akinetic rigid syndrome: An overview. Ann Indian Acad Neurol [online] 2007 December [viewed 20 September 2014] Available from: doi:10.4103/0972-2327.31481
  9. HOFFMAN O, WEBER RJ. Pathophysiology and Treatment of Bacterial Meningitis Ther Adv Neurol Disord [online] 2009 Nov, 2(6):1-7 [viewed 20 September 2014] Available from: doi:10.1177/1756285609337975
  10. TUNKEL ALLAN R., GLASER CAROL A., BLOCH KAREN C., SEJVAR JAMES J., MARRA CHRISTINA M., ROOS KAREN L., HARTMAN BARRY J., KAPLAN SHELDON L., SCHELD W. MICHAEL, WHITLEY RICHARD J.. The Management of Encephalitis: Clinical Practice Guidelines by the Infectious Diseases Society of America. CLIN INFECT DIS [online] 2008 August, 47(3):303-327 [viewed 20 September 2014] Available from: doi:10.1086/589747
  11. MARX GRACE E., CHAN EDWARD D.. Tuberculous Meningitis: Diagnosis and Treatment Overview. Tuberculosis Research and Treatment [online] 2011 December, 2011:1-9 [viewed 20 September 2014] Available from: doi:10.1155/2011/798764
  12. RAJASINGHAM RADHA, ROLFES MELISSA A., BIRKENKAMP KATE E., MEYA DAVID B., BOULWARE DAVID R., FARRAR JEREMY. Cryptococcal Meningitis Treatment Strategies in Resource-Limited Settings: A Cost-Effectiveness Analysis. PLoS Med [online] 2012 September [viewed 20 September 2014] Available from: doi:10.1371/journal.pmed.1001316
  13. MCKINNEY P A. Brain tumours: incidence, survival, and aetiology. Journal of Neurology, Neurosurgery & Psychiatry [online] 2004 June, 75(suppl_2):ii12-ii17 [viewed 20 September 2014] Available from: doi:10.1136/jnnp.2004.040741