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Levodopa is the gold standard for symptomatic treatment of Parkinson's disease (PD).
The salient feature of PD is the progressive deterioration of disability and increasing dependence.
A variety of drugs and therapies can slow the progression of disability and control symptoms to a certain extent; unfortunately, there is currently no accepted treatment.
This article brings you 7 PD cases and a simple quiz for diagnosis and treatment problems.
Come and try your skills.
Case 1 A 67-year-old man with 7 years of PD has increased urinary frequency and urgency.
Urinalysis and culture results are unclear.
Ultrasound images of the kidneys, ureters, bladder, and prostate are also normal.
Which of the following is most likely to cause these symptoms? A.
Benign prostatic hyperplasia B.
Urinary tract infection C.
Autonomic nerve dysfunction D.
Kidney stones As mentioned earlier, PD is mainly a motor dysfunction, traditionally characterized by tremor, bradykinesia and muscle rigidity.
As a neurodegenerative disease, PD follows a gradual and long-lasting process, leading to severe disability.
However, another feature of this disease is a wide range of non-motor symptoms (see Figure 1), which almost every patient has.
These symptoms may begin as early as 12-14 years before the onset of motor symptoms.
Depression is the most common of these non-motor symptoms; other notable examples include cognitive impairment, sleep disorders, autonomic dysfunction (orthohypotension, constipation, and urinary dysfunction), hypoosmia, pain, and fatigue1.
These complications aggravate the severity of the disease and lead to a deterioration of the patient's quality of life.
Therefore, they must be properly evaluated and managed.
Figure 1 Answer: C.
Case 2 A 70-year-old man presented with a history of left shoulder pain and gait difficulty for about 1 year.
He cannot swing his left arm while walking, and it is difficult to operate the buttons with his left hand.
The patient reported that he had constipation and fell from the bed in his sleep 6 years before the onset of gait difficulty.
Based on the possible diagnosis, which of the following is the most likely imaging result for this patient? A.
Normal brain magnetic resonance imaging (MRI) B.
Dopamine transporter (DAT) imaging increased DAT C.
Contrast enhancement of the basal ganglia on brain MRI D.
MRI showed bilateral T2 hyperintensity in the white matter around the ventricle PD has been a clinical diagnosis for a long time, and until recently, there have been no diagnostic studies on the diagnosis of the disease.
Even today, the final diagnosis can only be made through an autopsy of the patient’s brain tissue.
Computerized tomography (CT) and MRI of the brain can usually show normal results to exclude treatable diseases that mimic PD, such as stroke, normal pressure hydrocephalus, Wilson disease, and tumors.
Brain MRI is particularly helpful in determining the position of electrode placement during PD surgery and ensuring the accuracy of placement.
The cross in Figure 2 marks the placement of the thalamic stimulator.
Positron emission computed tomography (PET) may show a decrease in fluorodopa.
Figure 2 Single-photon emission computed tomography (SPECT) technology has been approved in the United States for the assessment of PD symptoms.
DAT has the function of taking DA from the protruding gap.
DAT is highly specific to PD and can detect dopaminergic degeneration of the substantia nigra striatum.
Answer: A.
Case 3 A 62-year-old patient with an 8-year history of PD treatment with levodopa showed obvious end-of-dose and dyskinesia.
Although the initial response to the drug treatment was good, there was still significant reduction in efficacy and dyskinesia.
Upon examination, the patient had a mask-like face and obvious static tremor of both upper limbs, and did not develop muscle rigidity or cognitive impairment.
Which treatment should this patient consider now? A.
Deep Brain Stimulation (DBS) B.
Thalamotomy C.
Globotomy D.
Stem Cell Transplantation The surgical options for PD include thalamotomy, Globotomy and DBS.
All the levodopa response characteristics of PD can be improved with DBS; however, this method cannot cure or change the course of PD disease.
Patients with levodopa-responsive idiopathic Parkinson's disease for 5 years or more and experiencing disabling side effects should be considered as candidates for DBS.
Clinically, dementia and depression must be excluded.
The dose of medication may be reduced after surgery.
DBS is a stereotactic surgery in which electrodes are placed in the subthalamic nucleus or globus pallidus and connected to a pulse generator under the clavicle.
Electrical stimulation is used to overcome abnormal currents generated in the circuits involved in PD.
Answer: A.
Case 4: A 62-year-old woman suffered from motor retardation and rigid limbs for about 3 years.
Since she was diagnosed with PD, she has been taking levodopa-carbidopa treatment.
In the past 6 months, she has been suffering from hyperhidrosis and urinary incontinence.
During the examination, the patient had obvious orthostatic (positional) hypotension.
The T2-weighted image of the brain MRI showed a "cross bun" sign in the pons.
Which of the following is the most likely diagnosis? A.
Progressive supranuclear palsy (PSP) B.
Shy-Drager syndrome C.
Stroke D.
Central nervous system tuberculosis Parkinson’s superimposed syndrome is a group of diseases characterized by Parkinson’s disease, accompanied by additional signs and symptoms Symptoms such as dementia, early-onset falls, and eye signs.
Another typical finding is the poor response to standard anti-Parkinson's drugs such as levodopa and ropinirole.
There are five kinds of Parkinson's superimposed syndromes in clinic, each syndrome has its own characteristics: (1) Multiple System Atrophy (MSA), (2) PSP, (3) Parkinson's Disease-Dementia-Amyotrophic Lateral Sclerosis Syndrome Signs, (4) Cortical Basal Ganglia Degeneration (CBD), and (5) Lewy Body Dementia (DLB).
MSA has been used as a common term for a group of syndromes, including olive pontine cerebellar atrophy (OPCA), striatal substantia nigra degeneration (SND), and Shy-Drager syndrome.
According to the current consensus, MSA can be divided into two categories, MSA (MSA-P) with Parkinson's disease as the mainstay and MSA with cerebellum as the characteristic (MSA-C).
Shy-Drager syndrome is a combination of Parkinson's disease and autonomic dysfunction (eg, orthostatic hypotension or hyperhidrosis).
The T2-weighted image of the MRI of the brain of patients with MSA shows a cross-shaped (bread) sign in the axial view of the pons (see Figure 3).
The selective degeneration of the cerebellopontine tract leads to a high-intensity crossover, which leads to a similar cause to the well-known pastry sign.
Figure 3 Answer: B.
Case 5: A 65-year-old male patient has progressive deterioration of his body stiffness, repeated falls, visual impairment and slurred speech for more than 10 years.
At the time of examination, in addition to motor retardation and muscle rigidity, the patient was also accompanied by paralysis of reclining and vertical gaze.
His face is a masked face, and his speech is monotonous.
The protrusion between the eyebrows can be seen.
He has been using levodopa-carbidopa for 5 years, but no significant improvement has been seen.
The T1-weighted image (sagittal image) of the brain MRI shows the "hummingbird (or penguin)" sign.
Which of the following is the most likely diagnosis? A.
PSPB.
Stroke C.
Motor neuron disease D.
Paralysis PSP (also known as Steele-Olszewski-Richardson disease) is characterized by idiopathic PD, with obvious postural instability, axial muscle rigidity, and vertical gaze dysfunction (Especially looking down) and pseudobulbar palsy.
PSP patients do not respond well to PD treatment.
They have a unique appearance, masked face, reduced blinking, and rigid trunk muscles.
The brainstem appearance of the brain MRIT1-weighted image (sagittal plane) is similar to the appearance of a "hummingbird" (or a penguin, see Figure 4).
It can be seen that the midbrain is atrophy and the pons can be preserved.
Figure 4 Answer: A.
Case 6 is a 71-year-old man with memory loss and recurring hallucinations for 4 years.
Since being diagnosed with PD five years ago with bradykinesia and resting tremor, he has been taking medications every day, including levodopa-carbidopa and ropinirole for treatment.
His Mini Mental State Examination (MMSE) score is 14 points (out of 30 points).
MRI of the brain showed atrophy of the whole brain.
Taking rivastigmine can improve visual hallucinations and memory loss.
Which of the following is the most likely diagnosis? A.
Alzheimer's disease B.
Vascular dementia C.
Parkinson's disease dementia D.
Normal pressure hydrocephalus Dementia is another non-motor complication encountered by PD patients.
There are two types of dementia associated with PD: Parkinson's disease dementia (PDD) and Lewy body dementia (DLB).
In PDD patients, the symptoms and signs of PD are at least one year earlier than the onset of dementia.
In DLB, the onset of dementia occurs almost simultaneously with the onset of Parkinson's symptoms.
In PDD, brain MRI may show non-specific cortical atrophy.
Several different scales are used to diagnose and rate the severity of dementia.
Among these scales, MMSE (see Figure 5) is the most commonly used tool.
Figure 5 Answer: C.
Case 7 An 18-year-old male patient developed a manic episode, hand tremor and gait disturbance.
On examination, he had gear-like rigidity, rubbing-like tremor, and slight yellowing of the sclera.
Slit lamp examination revealed the presence of Kayser-Fleischer (KF) rings on the upper edge of the cornea on both sides.
Which of the following is a possible diagnosis? A.
Sydenham chorea B.
Wilson disease C.
Huntington chorea D.
Systemic lupus erythematosus Wilson disease usually appears in the early stage and is characterized by psychosis, neuropathy, and liver disease, alone or in combination.
This is one of the reasons for Parkinson's disease-like symptoms in young people.
The gait is irregular and intermittent, just like a puppet.
Each step is accompanied by different movements of the limbs, torso and neck, with twitching and twisting.
The following MRI brain T2-weighted image shows the "double panda sign" characteristic of Wilson disease.
The abnormality of the midbrain constitutes the "face of a giant panda" (left), and the second "mini panda" can be seen in the panda's high signal abnormality (right) (see Figure 6).
Figure 6 Answer: B.
Yimaitong compiled from: Parkinsonism: Not Just a Motor Problem.
Medscape.
The salient feature of PD is the progressive deterioration of disability and increasing dependence.
A variety of drugs and therapies can slow the progression of disability and control symptoms to a certain extent; unfortunately, there is currently no accepted treatment.
This article brings you 7 PD cases and a simple quiz for diagnosis and treatment problems.
Come and try your skills.
Case 1 A 67-year-old man with 7 years of PD has increased urinary frequency and urgency.
Urinalysis and culture results are unclear.
Ultrasound images of the kidneys, ureters, bladder, and prostate are also normal.
Which of the following is most likely to cause these symptoms? A.
Benign prostatic hyperplasia B.
Urinary tract infection C.
Autonomic nerve dysfunction D.
Kidney stones As mentioned earlier, PD is mainly a motor dysfunction, traditionally characterized by tremor, bradykinesia and muscle rigidity.
As a neurodegenerative disease, PD follows a gradual and long-lasting process, leading to severe disability.
However, another feature of this disease is a wide range of non-motor symptoms (see Figure 1), which almost every patient has.
These symptoms may begin as early as 12-14 years before the onset of motor symptoms.
Depression is the most common of these non-motor symptoms; other notable examples include cognitive impairment, sleep disorders, autonomic dysfunction (orthohypotension, constipation, and urinary dysfunction), hypoosmia, pain, and fatigue1.
These complications aggravate the severity of the disease and lead to a deterioration of the patient's quality of life.
Therefore, they must be properly evaluated and managed.
Figure 1 Answer: C.
Case 2 A 70-year-old man presented with a history of left shoulder pain and gait difficulty for about 1 year.
He cannot swing his left arm while walking, and it is difficult to operate the buttons with his left hand.
The patient reported that he had constipation and fell from the bed in his sleep 6 years before the onset of gait difficulty.
Based on the possible diagnosis, which of the following is the most likely imaging result for this patient? A.
Normal brain magnetic resonance imaging (MRI) B.
Dopamine transporter (DAT) imaging increased DAT C.
Contrast enhancement of the basal ganglia on brain MRI D.
MRI showed bilateral T2 hyperintensity in the white matter around the ventricle PD has been a clinical diagnosis for a long time, and until recently, there have been no diagnostic studies on the diagnosis of the disease.
Even today, the final diagnosis can only be made through an autopsy of the patient’s brain tissue.
Computerized tomography (CT) and MRI of the brain can usually show normal results to exclude treatable diseases that mimic PD, such as stroke, normal pressure hydrocephalus, Wilson disease, and tumors.
Brain MRI is particularly helpful in determining the position of electrode placement during PD surgery and ensuring the accuracy of placement.
The cross in Figure 2 marks the placement of the thalamic stimulator.
Positron emission computed tomography (PET) may show a decrease in fluorodopa.
Figure 2 Single-photon emission computed tomography (SPECT) technology has been approved in the United States for the assessment of PD symptoms.
DAT has the function of taking DA from the protruding gap.
DAT is highly specific to PD and can detect dopaminergic degeneration of the substantia nigra striatum.
Answer: A.
Case 3 A 62-year-old patient with an 8-year history of PD treatment with levodopa showed obvious end-of-dose and dyskinesia.
Although the initial response to the drug treatment was good, there was still significant reduction in efficacy and dyskinesia.
Upon examination, the patient had a mask-like face and obvious static tremor of both upper limbs, and did not develop muscle rigidity or cognitive impairment.
Which treatment should this patient consider now? A.
Deep Brain Stimulation (DBS) B.
Thalamotomy C.
Globotomy D.
Stem Cell Transplantation The surgical options for PD include thalamotomy, Globotomy and DBS.
All the levodopa response characteristics of PD can be improved with DBS; however, this method cannot cure or change the course of PD disease.
Patients with levodopa-responsive idiopathic Parkinson's disease for 5 years or more and experiencing disabling side effects should be considered as candidates for DBS.
Clinically, dementia and depression must be excluded.
The dose of medication may be reduced after surgery.
DBS is a stereotactic surgery in which electrodes are placed in the subthalamic nucleus or globus pallidus and connected to a pulse generator under the clavicle.
Electrical stimulation is used to overcome abnormal currents generated in the circuits involved in PD.
Answer: A.
Case 4: A 62-year-old woman suffered from motor retardation and rigid limbs for about 3 years.
Since she was diagnosed with PD, she has been taking levodopa-carbidopa treatment.
In the past 6 months, she has been suffering from hyperhidrosis and urinary incontinence.
During the examination, the patient had obvious orthostatic (positional) hypotension.
The T2-weighted image of the brain MRI showed a "cross bun" sign in the pons.
Which of the following is the most likely diagnosis? A.
Progressive supranuclear palsy (PSP) B.
Shy-Drager syndrome C.
Stroke D.
Central nervous system tuberculosis Parkinson’s superimposed syndrome is a group of diseases characterized by Parkinson’s disease, accompanied by additional signs and symptoms Symptoms such as dementia, early-onset falls, and eye signs.
Another typical finding is the poor response to standard anti-Parkinson's drugs such as levodopa and ropinirole.
There are five kinds of Parkinson's superimposed syndromes in clinic, each syndrome has its own characteristics: (1) Multiple System Atrophy (MSA), (2) PSP, (3) Parkinson's Disease-Dementia-Amyotrophic Lateral Sclerosis Syndrome Signs, (4) Cortical Basal Ganglia Degeneration (CBD), and (5) Lewy Body Dementia (DLB).
MSA has been used as a common term for a group of syndromes, including olive pontine cerebellar atrophy (OPCA), striatal substantia nigra degeneration (SND), and Shy-Drager syndrome.
According to the current consensus, MSA can be divided into two categories, MSA (MSA-P) with Parkinson's disease as the mainstay and MSA with cerebellum as the characteristic (MSA-C).
Shy-Drager syndrome is a combination of Parkinson's disease and autonomic dysfunction (eg, orthostatic hypotension or hyperhidrosis).
The T2-weighted image of the MRI of the brain of patients with MSA shows a cross-shaped (bread) sign in the axial view of the pons (see Figure 3).
The selective degeneration of the cerebellopontine tract leads to a high-intensity crossover, which leads to a similar cause to the well-known pastry sign.
Figure 3 Answer: B.
Case 5: A 65-year-old male patient has progressive deterioration of his body stiffness, repeated falls, visual impairment and slurred speech for more than 10 years.
At the time of examination, in addition to motor retardation and muscle rigidity, the patient was also accompanied by paralysis of reclining and vertical gaze.
His face is a masked face, and his speech is monotonous.
The protrusion between the eyebrows can be seen.
He has been using levodopa-carbidopa for 5 years, but no significant improvement has been seen.
The T1-weighted image (sagittal image) of the brain MRI shows the "hummingbird (or penguin)" sign.
Which of the following is the most likely diagnosis? A.
PSPB.
Stroke C.
Motor neuron disease D.
Paralysis PSP (also known as Steele-Olszewski-Richardson disease) is characterized by idiopathic PD, with obvious postural instability, axial muscle rigidity, and vertical gaze dysfunction (Especially looking down) and pseudobulbar palsy.
PSP patients do not respond well to PD treatment.
They have a unique appearance, masked face, reduced blinking, and rigid trunk muscles.
The brainstem appearance of the brain MRIT1-weighted image (sagittal plane) is similar to the appearance of a "hummingbird" (or a penguin, see Figure 4).
It can be seen that the midbrain is atrophy and the pons can be preserved.
Figure 4 Answer: A.
Case 6 is a 71-year-old man with memory loss and recurring hallucinations for 4 years.
Since being diagnosed with PD five years ago with bradykinesia and resting tremor, he has been taking medications every day, including levodopa-carbidopa and ropinirole for treatment.
His Mini Mental State Examination (MMSE) score is 14 points (out of 30 points).
MRI of the brain showed atrophy of the whole brain.
Taking rivastigmine can improve visual hallucinations and memory loss.
Which of the following is the most likely diagnosis? A.
Alzheimer's disease B.
Vascular dementia C.
Parkinson's disease dementia D.
Normal pressure hydrocephalus Dementia is another non-motor complication encountered by PD patients.
There are two types of dementia associated with PD: Parkinson's disease dementia (PDD) and Lewy body dementia (DLB).
In PDD patients, the symptoms and signs of PD are at least one year earlier than the onset of dementia.
In DLB, the onset of dementia occurs almost simultaneously with the onset of Parkinson's symptoms.
In PDD, brain MRI may show non-specific cortical atrophy.
Several different scales are used to diagnose and rate the severity of dementia.
Among these scales, MMSE (see Figure 5) is the most commonly used tool.
Figure 5 Answer: C.
Case 7 An 18-year-old male patient developed a manic episode, hand tremor and gait disturbance.
On examination, he had gear-like rigidity, rubbing-like tremor, and slight yellowing of the sclera.
Slit lamp examination revealed the presence of Kayser-Fleischer (KF) rings on the upper edge of the cornea on both sides.
Which of the following is a possible diagnosis? A.
Sydenham chorea B.
Wilson disease C.
Huntington chorea D.
Systemic lupus erythematosus Wilson disease usually appears in the early stage and is characterized by psychosis, neuropathy, and liver disease, alone or in combination.
This is one of the reasons for Parkinson's disease-like symptoms in young people.
The gait is irregular and intermittent, just like a puppet.
Each step is accompanied by different movements of the limbs, torso and neck, with twitching and twisting.
The following MRI brain T2-weighted image shows the "double panda sign" characteristic of Wilson disease.
The abnormality of the midbrain constitutes the "face of a giant panda" (left), and the second "mini panda" can be seen in the panda's high signal abnormality (right) (see Figure 6).
Figure 6 Answer: B.
Yimaitong compiled from: Parkinsonism: Not Just a Motor Problem.
Medscape.
