Akira Kurita

Impaired myocardial 123I-metaiodobenzylguanidine uptake in Lewy body disease: comparison between dementia with Lewy bodies and Parkinson's disease.

BACKGROUND Iodine-123-labeled metaiodobenzylguanidine (123I-MIBG) myocardial scintigraphy has been used to evaluate cardiac sympathetic denervation in Lewy body disease (LBD) including Parkinsons disease (PD) and dementia with Lewy bodies (DLB). Patients with LBD had marked reductions in cardiac MIBG accumulation, indicative of severe impairment of the cardiac sympathetic nervous systems. However, the differences in scintigraphy between DLB and PD have not been determined. OBJECTIVE To compare cardiac sympathetic function in early disease stage measured with 123I-MIBG scintigraphy between DLB and PD. METHODS 123I-MIBG myocardial scintigraphy was performed in 22 patients with early-stage DLB, 41 patients with early idiopathic PD and 15 normal control subjects who were matched for age and disease duration. The heart-to-mediastinum (H/M) ratio was calculated. RESULTS 123I-MIBG uptake of the myocardium was significantly lower in patients with early DLB than in controls. The mean value of H/M ratio in patients with DLB was significantly lower than those in patients with PD, independent of the Hoehn and Yahr stage. CONCLUSIONS Our findings suggest that cardiac sympathetic function in DLB is severely impaired even in the early disease stage.

The Beneficial Effect of Donepezil on Visual Hallucinations in Three Patients with Parkinson's Disease

Visual hallucinations (VHs) are common psychiatric symptoms in patients with long-standing Parkinsons disease (PD). Treatment with neuroleptics or withdrawal of anti-PD drugs may improve VHs but will worsen motor dysfunctions. The authors report on 3 patients with long-standing PD who were treated with the cholinesterase inhibitor donepezil for the treatment of VHs. Each received a daily dose of 5 mg of donepezil, after reducing or discontinuing anti-PD medications had failed to relieve the VHs. In 2 patients (patient 1, 2), donepezil decreased VHs without worsening motor dysfunctions. In addition, the cognitive status of patient 2 improved. In patient 3, donepezil also resolved VHs, but delusions developed during treatment. After discontinuing donepezil, delusions disappeared and VHs reappeared. Donepezil may ameliorate visual hallucinations in PD patients, but controlled, double-blind trials are necessary to further clarify the effect of this drug on VHs in PD. (J Geriatr Psychiatry Neurol 2003; 16:184-188).

Preserved myocardial [123I] metaiodobenzylguanidine uptake in autosomal recessive juvenile parkinsonism : first case report

A decrease in myocardial uptake of iodine‐123–labeled metaiodobenzylguanidine (123I‐MIBG) has been reported in idiopathic Parkinsons disease (PD) using 123I‐MIBG myocardial scintigraphy. However, the patient with autosomal recessive juvenile parkinsonism (AR‐JP), caused by the parkin gene, presented here showed normal 123I‐MIBG myocardial uptake, suggesting that AR‐JP is a distinct disease entity from PD. Although the clinical features of AR‐JP are sometimes quite similar to those of late‐onset idiopathic PD, 123I‐MIBG myocardial scintigraphy may be a powerful tool to differentiate PD from other parkinsonian syndromes, including AR‐JP.

Visual hallucinations and altered visual information processing in Parkinson disease and dementia with Lewy bodies

Visual hallucinations (VHs) are common in dementia with Lewy bodies (DLB) and Parkinsons disease with dementia (PDD), while auditory hallucinations are rare. To neurophysiologically investigate the pathophysiology of VHs in these disorders, we studied event‐related potentials (ERPs) of DLB, PDD, and Alzheimers disease (AD) patients. We compared visual and auditory ERP latencies among PDD patients with and without VHs (PDD‐H: 11, PDD‐N: 6), DLB patients (24), and AD patients (21). To elicit visual and auditory ERPs, a facial discrimination paradigm and a conventional auditory odd‐ball paradigm, respectively, were used. The mean visual P3 latencies in the PDD‐H and DLB groups were significantly longer than that in the AD group, while the mean auditory P3 latencies in all four patient groups were comparable. The mean visual P2 latencies in the PDD‐N, PDD‐H, and DLB groups were significantly longer than that in the control group. Our findings suggest that visual cognitive functions are selectively impaired in hallucinatory patients with DLB and PDD. VHs may be associated in part with predominant visual cognitive impairments attributable to PDD and DLB pathologies. Our findings also suggest that the impairments occur at the early stage of facial information processing.

Pramipexole-induced antecollis in Parkinson's disease.

We report a case of antecollis, or dropped head with Parkinsons disease (PD) induced by pramipexole, a nonergot dopamine agonist. An 80-year-old woman presented with progressively severe neck flexion, which developed within a few weeks of taking pramipexole at 3 mg/day. She had a disturbed gait and complained of difficulty in daily activity because of restricted visual field and severe stooped posture. Surface EMG showed disproportionate tonus of the neck muscles but needle EMG of the neck muscles was normal. Withdrawal of pramipexole resulted in immediate improvement; the patient could keep the head in natural position and walk normally. Pramipexole-induced antecollis may be serious, but is a reversible dystonia in patients with PD. Clinicians should be aware of such complication.

Changes in auditory P300 event‐related potentials and brainstem evoked potentials in diabetes mellitus

To investigate the influence of diabetes mellitus on higher cognitive functions electrophysiologically, we studied auditory P300 event‐related potentials (P300) in 40 NIDDM patients, taking into account wave I‐V latencies (I‐V) in auditory brainstem evoked potentials, clinical parameters and head MRI findings. Compared with 20 controls, diabetics had significantly longer P300 and I‐V latencies. P300 latencies in diabetics correlated with neither I‐V. HbA1, blood glucose levels, nor disease duration. Of the 13 diabetics investigated neuroradiologically, four had lacunar infarcts with prolonged electrophysiological values. The remaining nine had normal MRI scans, but their physiological parameters were still significantly longer than those of controls. These findings suggest that NIDDM can independently alter higher cognitive and the central auditory pathway functions. Our data also suggest that these alterations occur regardless of the recent metabolic derangement and disease duration. Cerebrovascular ischemia, if present, also appears to contribute in part to cognitive alterations.

Neurophysiological evidence for altered higher brain functions in NIDDM.

OBJECTIVE To investigate neurophysiological alterations of higher brain function in patients with NIDDM. RESEARCH DESIGN AND METHODS Auditory P300 event-related potentials were recorded in 60 NIDDM patients who had no evidence of stroke, dementia, or any other neurological illnesses. The P300 wave latencies in diabetic patients were compared with those in neurologically healthy control subjects, with consideration of clinical parameters and diabetic complications. RESULTS Diabetic patients had significantly longer P300 latencies than control subjects. Seven diabetic patients had pathologically prolonged P300 values. There was a trend toward longer P300 latencies in diabetic patients with retinopathy and in those with HbA1 of ≥10%; the differences between these diabetic subgroups and control subjects were both statistically significant. However, three of seven diabetic patients with abnormal P300 latencies had no retinal lesions, and no significant correlation was observed among diabetic patients between P300 latencies and HbA1 levels. Peripheral neuropathy, nephropathy, blood glucose levels, and disease duration appeared not to correlate with P300 alterations. CONCLUSIONS These findings suggest the presence of central nervous pathological processes in NIDDM that affect higher brain functions, as assessed by P300 latencies. Our findings also suggest that microangiopathy and metabolic derangement during the preceding 4- to 8-week period may contribute in small part to the pathophysiology of this central nervous involvement.

Metastatic renal cell carcinoma initially presented with a longitudinally extensive spinal cord lesion on MRI.

A 48-year old male patient developed numbness in the lower half of the body 5 months after the curative operation of left renal cell carcinoma. Neurological examinations revealed the sensory disturbance below the T10 dermatome. A sagittal T2WI of the spinal MRI demonstrated a longitudinally extensive spinal cord lesion (LESCL) ranging from the C7 to L1 vertebral level. The neurological deficits rapidly deteriorated to paraplegia with bladder bowel disturbance. The high dose steroid pulse therapy showed temporary effect. The Gd enhanced T1WI of the spinal MRI taken on the 24th hospital day demonstrated a solitary intramedullary mass in the T8-9 level with ring enhancement, and a subsequent total resection of the tumor was performed. The histopathological studies of the tumor lead to the diagnosis of intramedullary spinal cord metastasis of the renal cell carcinoma. The post-operative T2WI of the spinal MRI revealed disappearance of the longitudinally extensive lesion. On the 112 hospital day, he was discharged with ambulatory aid. While solitary intramedullary spinal cord metastasis of renal cell carcinoma is quite rare, it should be suspected when the LESCL is revealed on a spinal MRI, even after the curative operation of the primary tumor.

難治性顔面痛の病態として，small fiber neuropathyの関与が強くうたがわれた完全型Heerfordt症候群の1例

We report a case of complete Heerfordt syndrome accompanied by the involvement of small fiber neuropathy (SFN) manifesting as refracory facial pain. A 30-year-old man presented with pyrexia, a 2-week history of facial burning pain, and difficulty of mastication. After admission to our hospital, neurological examinations showed bilateral facial pain, trigeminal motor palsy, left facial nerve palsy, bilateral sensory neural deafness, uveitis and swelling of the parotid gland. Other examinations revealed bilateral hilar lymphadenopathy, high serum titer of angiotensin coenzyme, and no response in a tuberculin-tested, non-caseating epithelioid granuloma from lip biopsy, leading to the diagnosis of complete Heerfordt syndrome. Mandibular skin biopsy with immunostaining for PGP 9.5 showed SFN. High-dose corticosteroids proved somewhat effective against SFN as facial pain, but reducing the corticosteroid dose proved difficult, as symptoms were refractory to other immunosuppressants and pain-control drugs such as anti-epileptics and anti-depressants. The patient died of acute pancreatitis 3 years after disease onset. Autopsy showed no granuloma in hilar lymph node, trigeminal nerve, cranial base, nerve root, and muscle. SFN in this case probably represent a cause of refractory facial pain.

P34.4 Masked face in Parkinson’s disease is related to decreased blink rate

Masked face, a well-known sign of Parkinson’s disease (PD), generally refers to lack of facial expression, a phenomenon often associated with decreased blink rate. We studied the relation between blink rate and various conditions, such as expression, akinesia and disease severity, in patients with PD. The subjects were 14 healthy controls (mean age: 67.6 years) and 52 patients with PD (70.1 years). Blink rates per minute was measured two times with the subjects in a sitting position. The results are as follows.