Hiroaki Fujita

Evaluation of cutoff scores for the Parkinson's disease sleep scale-2

The Parkinsons Disease Sleep Scale (PDSS)‐2 is a recently developed tool for evaluating disease‐related nocturnal disturbances in patients with Parkinsons disease (PD). However, its cutoff score has not been clinically assessed. We determined the optimal cutoff score of the Japanese version of the PDSS‐2.

Usefulness of Cardiac MIBG Scintigraphy, Olfactory Testing and Substantia Nigra Hyperechogenicity as Additional Diagnostic Markers for Distinguishing between Parkinson’s Disease and Atypical Parkinsonian Syndromes

Background We aimed to evaluate the utility of the combined use of cardiac 123I-metaiodobenzylguanidine (MIBG) scintigraphy, olfactory testing, and substantia nigra (SN) hyperechogenicity on transcranial sonography (TCS) in differentiating Parkinson’s disease (PD) from atypical parkinsonian syndromes (APSs), such as multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). Methods Cardiac MIBG scintigraphy, card-type odor identification testing (Open Essence (OE), Wako, Japan), and TCS were performed with 101 patients with PD and 38 patients with APSs (MSA and PSP). Receiver operating characteristic (ROC) curve analysis was used to assess the sensitivity and specificity of these batteries for diagnosing PD from APSs. The diagnostic accuracy of the three tests was also assessed among patients at the early disease stage (drug-naïve patients with a disease duration of 3 years or less). Results In differentiating PD from APSs, the area under the ROC curve was 0.74 (95% CI, 0.65–0.83), 0.8 (95% CI, 0.73–0.87), and 0.75 (95% CI, 0.67–0.82) for TCS, cardiac MIBG scintigraphy, and olfactory testing, respectively. The diagnostic sensitivity and specificity were 53.1% and 91.7%, respectively, for TCS, 70.3% and 86.8%, respectively, for cardiac MIBG scintigraphy, 58.4% and 76.3%, respectively, for OE. Among early-stage patients, sensitivity and specificity were 50.0% and 93.8%, respectively, for TCS, 57.1% and 87.5%, respectively, for cardiac MIBG scintigraphy, and 54.8% and 79.2%, respectively, for OE. At least one positive result from 3 tests improved sensitivity (86.1%) but decreased specificity (63.2%). In contrast, at least 2 positive results from 3 tests had good discrimination for both early-stage patients (50.0% sensitivity and 93.8% specificity) and patients overall (57.8% sensitivity and 95.8% specificity). Positive results for all 3 tests yielded 100% specificity but low sensitivity (25%). Conclusions At least 2 positive results from among TCS, cardiac MIBG scintigraphy, and olfactory testing can support clinical diagnosis in distinguishing PD from APSs.

Serum uric acid levels in Parkinson's disease and related disorders

Serum uric acid (UA) levels are reported to be decreased in patients with Parkinsons disease (PD) and multiple system atrophy (MSA). However, clinical correlates of serum UA levels are still unclear in PD‐related disorders. We conducted a cross‐sectional study to evaluate the associations between serum UA levels and disease duration, disease severity, and motor function among PD, MSA, and progressive supranuclear palsy (PSP) patients.

Sleep Disturbances in Patients with Parkinson’s Disease

Sleep disturbances are among the most common disabling nonmotor symptoms of Parkinson disease (PD). Recently, awareness of sleep disorders related to PD has increased, reflecting their significant negative impact on the quality of life of the patient. However, sleep disturbances are still often under-recognized. In PD, multiple factors are associated with sleep disturbances; impaired arousal systems and sleep structure as disease-related changes, nocturnal motor symptoms, hallucinations and psychosis, pain, nocturia, depressive symptoms and dopaminergic medication can all cause insomnia. Further, these factors can coexist and influence each other during the course of the disease. In addition, sleep-related breathing disorder, restless legs syndrome (RLS) and rapid eye movement sleep behavior disorder (RBD) can also lead to impaired sleep. Some idiopathic RBD patients have been associated with a risk for developing neurodegenerative diseases, including PD. Therefore, early diagnosis of RBD may have significant clinical implications for neuroprotective strategies. Several studies have revealed an increased incidence of RLS in PD patients compared to the general population. The favorable responses to dopaminergic medications in treating both RLS and PD suggest shared pathophysiologies between the two diseases; however, neuroimaging studies of dopaminergic systems remain inconclusive. In this review, we discuss the current understanding of sleep disturbances in PD and the existing treatments.

A Card-type Odor Identification Test for Japanese Patients with Parkinson's Disease and Related Disorders

Objective The characteristics of olfactory impairment in Parkinsons disease (PD), multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) have not been determined in detail. We assessed the olfactory function among PD, MSA and PSP patients. Methods A card-type odor identification test, Open Essence (OE, Wako, Japan), which consists of 12 different odorants familiar to Japanese subjects, was administered to 98 PD patients, 32 MSA patients, 17 PSP patients and 96 control subjects ≥50 years of age. Results The PD patients had significantly lower OE scores than the other groups. The OE scores of the MSA and PSP patients fell between those of the PD patients and the control subjects. A cut-off OE score of 6 was beneficial for differentiating PD patients from controls with 84.7% sensitivity and 85.4% specificity. A cut-off OE score of 4 had 60.2% sensitivity and 77.6% specificity for differentiating PD patients from MSA and PSP patients. The correct answer rates for the curry, Japanese orange and perfume odorants in the PD patients were lower than those in the MSA and PSP patients and controls. The PD patients also had the highest ratio of “not detected” choices across the 12 odors. Conclusion Marked olfactory impairment was a feature of the patients with PD, while mild olfactory impairment was observed in those with MSA or PSP. The answer patterns and the specific odorants may also be useful in differentiating PD from related disorders.

Resting and re-emergent tongue tremor in Parkinson’s disease

An 82-year-old man was referred to our department for re-evaluation of parkinsonism. The patient had a 2-year history of parkinsonism followed by dementia and was treated with 300 mg of levodopa/decarboxylase inhibitor. The patient previously had a significant response of motor symptoms including rest and re-emergent tremor to levodopa therapy. The medical history included chronic subdural haematoma. Neurological examination showed bradykinesia and rigidity involving the neck and upper and lower extremities. Resting tremor at the jaw, tongue and right foot was observed. Re-emergent …

Demyelinating hypertrophic inferior alveolar nerve mimicking a nerve tumor.

We herein report a patient with demyelinating inferior alveolar nerve hypertrophy, which was initially suspected to have a nerve tumor. A 39-year-old woman with childhood-onset polyneuropathy presented with tooth pain and visited a dental clinic. An X-ray examination of the mandible revealed enlargement of the mandibular canal, and a nerve tumor was suspected. CT scan and MRI showed hypertrophy of the inferior alveolar nerve along its entire length. We diagnosed the patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which was supported by the spontaneous recovery reported in her childhood, the results from a nerve conduction study and MRI data. CIDP should be considered in the differential diagnosis of mandibular canal enlargement.

Increased frequency and spread of restlessness as the early manifestation of Parkinson’s disease in a woman with restless legs syndrome

A 57-year-old woman had been suffered from insomnia due to restlessness and abnormal sensation of the left side of the body for 33 years. Since the preceding year of the first visit frequency of the symptoms increased, and the abnormal sensation was spread to the right leg. Her daughter had restless legs syndrome (RLS) since age 20. Neurological examination showed no abnormality. Laboratory test results showed normal ferritin levels. There was no renal dysfunction or anemia. A diagnosis of RLS was made, and her symptoms responded well to pramipexole treatment. However, the patient developed right shoulder pain and right-hand tremor one year and one and a half year after the first visit, respectively. Based on clinical findings and the findings of dopamine transporter scan and cardiac 123I-MIBG scintigraphy, the patient was diagnosed with Parkinsons disease (PD). Careful observation of changes in RLS symptoms is required as an increased frequency and spread of symptoms of RLS could be the early manifestation of PD.

Restless legs syndrome, leg motor restlessness and their variants in patients with Parkinson's disease and related disorders

OBJECTIVE The objective of this study was to investigate the prevalence of restless leg syndrome (RLS), leg motor restlessness (LMR) and RLS/LMR variants and their relationship with clinical factors in patients with Parkinsons disease (PD) and related disorders. METHODS Sixty-three PD patients, 17 multiple system atrophy (MSA) patients and 11 progressive supranuclear palsy (PSP) patients were included in this study. Through face-to-face interviews, the patients were diagnosed with RLS/LMR, or with RLS/LMR variants in which the symptoms occur predominantly in body parts other than the legs. RESULTS The frequency of RLS, LMR, RLS variants and LMR variants was as follows: PD (12.7%, 11.1%, 0% and 1.6%); MSA (5.9%, 11.8%, 0% and 0%); and PSP (0%, 9.1%, 0% and 0%). Restlessness without the urge to move was observed in 25.4% of the PD patients, 11.8% of the MSA patients and 0% of the PSP patients. The PD patients with restlessness exhibited higher Hoehn and Yahr stages and higher scores on the Scales for Outcomes in PD-Autonomic, PD sleep scale-2 and Beck Depression Inventory-II. The olfactory functioning, 123I-MIBG myocardial scintigraphy uptake and dopamine transporter single photon emission computed tomography findings did not differ between the PD patients with restlessness and those without. The severity of RLS was correlated with the autonomic symptoms among the PD patients with restlessness. CONCLUSION PD with restlessness was characterized by increased autonomic, sleep and depressive symptoms. Further studies including a large sample are warranted to characterize restlessness in PD and related disorders.

Isolated pontine involvement of posterior reversible encephalopathy syndrome with concomitant ischemic cerebral infarction

In posterior reversible encephalopathy syndrome, isolated involvement of the brainstem is rare. In addition, there have been a few reports describing a concomitant occurrence of stroke and a brainstem variant of posterior reversible encephalopathy syndrome. We here report a patient with isolated pontine involvement of posterior reversible encephalopathy syndrome accompanied by fresh ischemic cerebral infarction in the left corona radiata detected by brain magnetic resonance imaging. Follow‐up brain magnetic resonance imaging on hospital day 14 showed a marked improvement of signal changes in the pons; however, cerebral infarction in the left corona radiata enlarged despite medical treatment. Although rare, clinicians should be aware of the co‐occurrence of posterior reversible encephalopathy syndrome and ischemic infarctions, especially in patients with untreated/uncontrolled hypertension and chronic kidney disease. We discuss the possible mechanism related to brainstem posterior reversible encephalopathy syndrome and stroke, reviewing the previous case reports.