Rest tremor
Freezing
Rigidity
Freezing
Bradykinesia
Festination
Reptile stare
Abnormal posture
Masked face
Camptocormia
Small voice
Pisa syndrome
Dysphagia
Head drop
Bradykinesia
Loss of automatic acts
Micrographia
Drooling
Shuffling gait
Aspiration
Postural instability
Loss of arm swing
Retro-, ante-, latero-pulsion
Loss of two motor acts
Stooped posture
8.3.3 Tremor
Presenting symptom in PD is in about 50 % unilateral rest tremor in the hand or the leg, mostly in the hand. But in DLB tremor is less frequent than in PD. In the clinical course, 75–80 % of PD patients will develop tremor. The tremor in PD is in most cases rest tremor. The tremor is between 4 and 6 cycles per second. The tremor is most often seen while the patient is walking or sitting. When the patient moves his or her shaking hand or taking the posture such as stretching arms in front, the tremor will stop or decrease its severity. But occasionally stretching the arms in front will induce much larger and prominent tremor; there is a brief period (approximately less than 10 s) of tremor ceasing. The tremor will become much larger and vigorous as long as the patient takes the arm-stretched posture, and often the tremor is asymmetric. Such tremor is called as reemergent tremor, and the presence of reemergent tremor indicates the clinical diagnosis of PD [10], although the reemergent tremor is rare in PD.
8.3.4 Rigidity
Rigidity is a form of increase in the muscle tone. When a muscle is passively stretched slowly, for instance, flexing and extending the wrist joint on one side, the examiner feels a resistance to the flexion and the extension in rigidity. This resistance is rather constant at all the flexed or extended period. This is a differentiating point from spasticity where the resistance is strong in the beginning but will soon melt away as the flexion or extension is continued. This is called a clasp-knife phenomenon in spasticity. In rigidity, sometimes the resistance is not continuous but noncontinuous interspersed with a tremor-like rhythm. This is called cogwheel rigidity and is usually seen in the upper extremity examination in PD, at times also in the lower extremity examination. Truncal rigidity can be tested by flexion of the neck in a supine position or rotating the trunk to the right and left in a sitting or a standing position.
8.3.5 Bradykinesia
Bradykinesia denotes slowness of movement. Every movement in PD becomes slow. Akinesia denotes loss of spontaneous or automatic movement. For instance, a PD patient sitting in a chair for a certain period may not move his or her hands or legs spontaneously, or there may be a marked reduction in such a spontaneous movement. Hypokinesia denotes smallness of movements. Any movement becomes smaller than normal. At times, the term bradykinesia is used to inclusively denote bradykinesia, akinesia, and hypokinesia. To exam whether or not the patient has bradykinesia, just watching the patient when he or she enters the examining room is suffice. The patient may walk slowly, may shuffle the legs, have no arm swing, have a stooped posture, have a loss of facial expression (masked face), have a reduced eye blinking, and have a reduced searching eye movement (reptile staring). Frequently, the patient has an asymmetric presentation of these symptoms; usually the side where the disease started is more involved than the other.
The voice becomes small, monotonous, and poor in intonation. At times, the pitch of the voice becomes higher than before the patient was afflicted with parkinsonism. The speech ultimately becomes mumbling and unintelligible difficult to understand. Handwriting is also affected. Initially, the size of the letter is not small but the patient continues writing; the size of the letter becomes smaller and smaller. This is called micrographia. When the patient walks, usually the arm swing is diminished or lost.
In examination of bradykinesia, we usually ask the patient to do finger tapping, rapid alternating movement, opening and closing the hands, tapping the floor with the heel with the knee up in the air as possible (foot agility), and tapping the floor with the tip of toes. In these tests, the rate of the speed in repetitive movements is reduced and the size of the movement is small. Each of these movements should be tested one extremity at one time; otherwise, subtle asymmetry of the movement may be missed.
8.3.6 Postural Instability
Postural instability can be tested by the pull test. In the test of retropulsion, ask the patient to stand up with feet apart at about the shoulder’s width. Then, the examiner stands behind the patient and explains to the patient what is going to happen. Then the examiner put his or her both arms on the shoulders of the patient. Then the examiner pulls the shoulders of the patient backward with the strength that will induce a step backward in a normal person. If the patient steps backward more than two steps or would fall backward if unsupported, the patient is said to have retropulsion. At times, the patient would fall backward without stepping even one step. In a similar way, ante-pulsion and latero-pulsion can be tested for the examiner standing in front of the patient.
8.3.7 Abnormalities in Posture
The posture of PD shows a stooped posture. The patient shows a bend forward posture mainly in the upper part of the thoracic spine. Perhaps this is a compensatory posture that the patient has a tendency to fall backward. The thoracolumbar region is straight in uncomplicated PD. If this part takes a bend forward posture upon standing, it is called camptocormia. The cause of camptocormia is not well known. It may be a dystonic posture originating from the brain of PD patients. At times, a focal myositic lesion is found in the paravertebral region [11]. But this myositic change is believed to be a secondary phenomenon due to a long-standing flexed posture. Camptocormia may be seen as an untoward symptom of dopamine agonists, particularly of non-ergot dopamine agonists [12]. The head drop or ante-collis is a strong anteflexion of the head so that the chin is almost touching the chest wall. This is more common in multiple system atrophy than in PD. The cause is not known. Pisa syndrome is a bend of the spine toward the right or left. Slight Pisa syndrome is frequently seen in PD. Pisa syndrome is thought to be a result of dystonia. But at times, dopamine agonists may induce Pisa syndrome [13].
8.3.8 Gait
Gait disorder in PD is a result of bradykinesia and postural instability. The typical feature of gait disorder in a moderately advanced PD patient takes a form of stooped postured, shuffling gait without arm swing looking downward with flexed neck. Some asymmetrical features may be soon. For instance, arm swing and shuffling gait is less involved on one side of the body. The patient is able to walk up and down the staircase rather easily.
8.3.9 Freezing
Freezing of gait is seen in moderately advanced PD patients. While walking, suddenly the feet are routed to the ground. The patient is unable to raise legs. Meanwhile the upper half of the body would move forward, and the bend forward posture will become extreme so that the patient must fall down to the floor. This freezing tends to occur more frequently within the house rather than outside of the house. Narrow places such as a toilet, a corner of the corridor, or a kitchen tend to precipitate freezing. Also, it can happen in front of the chair that he or she wants to sit down and the patient may fall down to the chair without moving feet.
Outside of the house, freezing of gait can occur, but it is more frequently in the form of festination. Here the patient with PD, while walking, the stride becomes smaller and smaller and the pace of walking is getting faster and faster. At the same time, the stooped posture will become prominent more and more, and ultimately the patient has to hold such an object as an electric pole to stop; otherwise, the patient may fall down to the ground. Both freezing and festination occur more frequently during off period compared to on period of levodopa intake, but it may also occur during on period. Therefore, dopamine deficiency is the most important mechanism for the freezing and the festination, but unknown additional mechanism must be working, as they may happen during on period of levodopa treatment.
8.3.10 Loss of Automatic Movements
Moderately advanced PD patients may have drooling. The saliva unconsciously comes out of the mouth, particularly when the patient is engaged in other acts such as watching TV, walking, or eating. The amount of the saliva is not increased in PD. Normal persons unconsciously swallow the saliva. This unconscious act of saliva swallowing is difficult for PD patients. Difficulties of unconscious motor acts can be seen frequently in daily life. For instance, loss of arm swing seems to be one example. Gait tends to become shuffling when the patient does not consciously pay attention to his or her feet. Aspiration is another example of loss of automatic movement.
8.3.11 Loss of Two Motor Acts
The patient with PD has a difficulty in doing two independent motor acts simultaneously in each hand. For instance, drinking a cup of coffee while reading a newspaper in the breakfast may be difficult to do. Another example is carrying a tray with dishes of soup to the dining table from the kitchen; this patient may fall down during the way. She has to pay attention to the arms so that she would not spill the soup; her attention to the feet becomes weak and she would fall down by stumbling to some object. For PD patient, it is important to do one motor act at one time.
8.3.12 Motor Fluctuations
In PD, usually the patients are doing well for the first 5 years after starting levodopa therapy without severe motor fluctuations such as wearing off or dyskinesia. After 5 years, those patients start to have motor fluctuations, and after 10 years of treatment with levodopa, more than 60 % of the patients are facing wearing off, and this percentage is becoming larger as the treatment period prolongs. Frequently on time from levodopa may last only 3 h or less and these patients may have to take levodopa six times or more to keep them in on time. Still they have on and off fluctuation. Furthermore, about 50 % of the levodopa-treated patients for more than 10 years may suffer from on-time dyskinesia. The patients may not notice a mild dyskinesia, but caregivers notice even a mild dyskinesia and feel uneasy. In a recent article from China [14], wearing off after 10 years of levodopa treatment was reported to be 68.3 % and dyskinesia 19.3 %. According to our unpublished data, the prevalence of wearing off was 81.7 % in patients with 10–15 years from the onset and dyskinesia 63.3 %.
8.4 Parkinsonism in DLB
In DLB, the rest tremor is less encountered than PD. In DLB, the rest tremor is seen in about 25 % of the patients. This difference may in part due to the difference in age of onset between PD and DLB. The age of onset in PD is usually between 55 and 70, while that of DLB is usually 65 or above. Burn et al. [15] studied clinical symptoms of parkinsonism in PD, PDD, and DLB. The mean age of onset was 67.4 in PD and PDD combined and 74.6 in DLB. Clinical dichotomy of PD into two forms is accepted by most of the movement disorder specialists. Jankovic and Kapadia [16] classified PD into two forms: tremor-dominant form and postural instability-gait disturbance (PIGD) form. The tremor-dominant form tends to progress more slowly than the rigid-akinetic form [16]. Dementia is more frequent in the rigid-akinetic form. Burn et al. [15] studied 38 PD, 43 PDD, and 26 DLB patients for subtypes of parkinsonism. Postural instability-gait difficulty (PIGD) subtype was more common in the PDD (88 % of cases) and the DLB (69 % of cases) groups compared with the PD group (38 % of cases). They also reported that cognitive decline in 2-year period was greater in the PIPG subtype [17].
Other difference is in the frequency of motor fluctuation. Here, again the age of onset seems to have an influence on the frequency of motor fluctuation. Frequencies of motor fluctuations tend to be higher in PD patients with young age of onset. In DLB, no such report is available on motor fluctuations with levodopa treatment. Perhaps, lower frequencies of motor fluctuation in DLB as compared with PD may be due to the higher age of onset, due to the shorter period of levodopa treatment in DLB, and due to dementia and hallucination, which may be limiting factors in increasing the dosage of levodopa. In PD, duration of levodopa treatment and the age of onset are important factors for motor fluctuations. Schrag and Quinn [18] reported incidence of wearing off to be 40 % and that of dyskinesia 28 % in a community-based population of PD, and the total number of PD was 124. What they found was those PD patients with wearing off were 68.8 years in average and the years of treatment with levodopa was 7.4 years, while those without wearing off had an average age of 73.3 years and the years of treatment with levodopa was 3.5 years. Thus, younger age of onset of PD and the longer duration of levodopa treatment tend to induce wearing-off phenomenon. The higher age of onset of DLB seems to be a factor for lower incidence of wearing off; however, the frequency of wearing off and dyskinesia has to be investigated in DLB.
Otherwise, parkinsonism in PD and parkinsonism in DLB are essentially similar.
8.5 Non-motor Symptoms in PD and in DLB
Many non-motor symptoms are known in PD and DLB (Table 8.2).
Table 8.2
Non-motor symptoms of parkinsonism and dementia with Lewy bodies
Autonomic disturbances | Disturbance of affect |
Constipation | Anxiety |
Nocturnal urinary frequency | Depression |
Impotence | Apathy |
Orthostatic hypotension | Sensory symptoms |
Postprandial hypotension | Hyposmia |
Edema | Pain |
Sweating | Loss of taste sensation |
Sleep disorders | Fatigue |
Insomnia | ICD and DA dysregulation syndrome |
REM sleep behavior disorder | Hallucination, delusion |
Restless legs syndrome | Violent behavior, hypersexual behavior, psychosis |
Disturbance of awakening | Dementia |
Excessive daytime sleepiness | |
Sudden onset of sleep |
8.5.1 Autonomic Disorders
The initial symptom in PD and DLB is in most cases constipation. The onset of constipation may precede the onset of parkinsonism or dementia for years or more than 10 years. In addition to constipation, diverse symptoms of autonomic disturbances frequently occur in PD and DLB including nocturnal polyuria, sexual disturbances, hypotension and orthostatic hypotension, postprandial hypotension, swinging blood pressure, profuse sweating, pretibial edema, and so on. As the pathology of PD and DLB seems to start in the peripheral autonomic nerves including the dorsal motor nuclei of the vagal nerves and climb up in the brain stem eventually involving the cerebral cortices [19], autonomic dysfunctions seem to be the first symptom of PD and DLB. Autonomic dysfunctions in DLB are covered in another chapter of this book.
8.5.2 Sleep Disorders
8.5.2.1 Insomnia
Difficulty in falling asleep, frequent awakenings during night, and difficulty in falling asleep toward the early morning are the causes of insomnia. Depression, anxiety, pain in some place, difficulty in rolling over, dystonia, and urinary frequency including prostate hypertrophy or cancer may become a cause of difficulty in falling asleep. Difficulty in falling asleep in uncomplicated PD patients is not very common. However, more frequently difficulty of falling asleep after wakening up in the midnight is encountered in PD. Difficulty in falling asleep should be treated properly. One may use a sleeping pill which has to be taken 30 min before the patient wants to asleep. Short-acting sleeping pills such as zolpidem (5–10 mg), zopiclone (7.5–10 mg), brotizolam (0.25 mg), or triazolam (0.125– 0.25 mg) are recommended.
The most frequent cause for awakening during night is urinary urgency. The urinary urgency occurs for both men and women. This probably due to lesion in the parasympathetic innervation to the detrusor muscles of the bladder. Secondary causes for frequent awakening during night include prostate hypertrophy or cancer, dystonia, pain, difficulty in rolling over, and spasm of the legs. For men, when frequent awakening takes place, prostate cancer should be ruled out by examination of prostate antigen in the blood; if the prostate antigen is more than 4, the patient should be introduced to an urology specialist. For frequent awakening during night, one may use a longer sleeping pill such as flunitrazepam (0.5–2 mg), nitrazepam (5–10 mg), estazolam (1–4 mg), or nimetazepam (3–5 mg) before sleep, or the very short-acting sleeping pill, triazolam (0.125– 0.25 mg), can be given when the patient wakes up in the midnight and complains of difficulty in falling asleep.
8.5.2.2 REM Sleep Behavior Disorder
When people have rapid eye movement (REM) sleep, usually he or she is silently sleeping without any movement in the limbs or in the vocalization. Muscle tones are hypotonic. He or she is dreaming without moving limbs or vocalization. The content of the dream is usually not scary. In the central nervous diseases such as PD, DLB, or MSA where the substantia nigra is involved, muscle tone will not disappear during REM sleep. The patient may be acting out the content of the dream. The dream is usually a scary one. The patient may shout or vigorously move his or her arms or legs as if he or she may be trying to fight or escape the scary content of the dream. The patient usually does not remember what he or she was doing during night. But at times, the patient may be awakening by the voice. Usually RBD appears before the motor symptoms of parkinsonism. Frequency of RBD in PD is reported as 30 % or above [20] and 60 % or above in DLB [21, 22]. The responsible lesion for RBD is not well elucidated yet; however, the sub-locus coeruleus area is thought to be responsible in PD [23]. Therefore, this structure is considered to be involved in PD and DLB before the substantia nigra is involved. RBD is thought to be a symptom, which appears earlier than motor symptoms of parkinsonism. RBD may be a prodromal symptom of other CNS disorders such as multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, or Alzheimer’s disease [24]. However, frequencies of RBD in other disorders are low except for multiple system atrophy [25].
Related posts:
Cognitive Impairments of Dementia with Lewy Bodies
Clinical Diagnostic Criteria for Dementia with Lewy Bodies
History and Latest Concepts of Lewy Body Disease and Dementia with Lewy Bodies
Molecular Biology of Dementia with Lewy Bodies
123I-Metaiodobenzylguanidine Myocardial Scintigraphy in Dementia with Lewy Bodies
Autonomic Symptoms in Dementia with Lewy Bodies
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