Dementia in Parkinson's disease: diffusion tensor imaging.

OBJECTIVE: Dementia occurs frequently in patients with Parkinson's disease (PD). However, the nature of the dementing process remains controversial. We evaluated various cognitive functions in patients with PD, compared fractional anisotropy (FA) values between PD patients with and without dementia. METHODS: Thirty-seven consecutive patients with Hoehn-Yahr stage III or IV PD participated in this study. Patients were divided into two groups: (i) PD with dementia group (PDD) and (ii) PD without dementia group (PDND). There were 11 PDD and 26 PDND cases. Ten controls were also studied. RESULTS: The PDD group showed significant FA reduction in the bilateral posterior cingulate bundles compared with PDND. FA values in the left posterior cingulate bundle showed significant correlations with many cognitive parameters. INTERPRETATION: Our results showed that the posterior cingulate areas play some important roles in the dementing process in PDD. However, as the pathological processes responsible for dementia in PD patients may be multifaceted, further studies are necessary.

Wisconsin Card Sorting Test in Parkinson's disease: diffusion tensor imaging.

INTRODUCTION: It is generally assumed that executive dysfunctions in Parkinson's disease (PD) are caused by degeneration of the basal ganglia or frontal cortex or both. However, there have been few studies investigating the relationship between executive dysfunctions and cerebral pathological change. The objective of this study was to evaluate various cognitive functions in non-demented patients with PD, and to compare the fractional anisotropy (FA) values of PD patients with and without executive dysfunction. MATERIALS AND METHODS: Twenty-one consecutive non-demented patients with PD were enrolled in this study. Patients were divided into two groups on the basis of their Wisconsin Card Sorting Test score. RESULTS: There was significant FA reduction in the left parietal white matter in the group in which the number of categories achieved was 2. CONCLUSION: Accumulating evidence suggests that conventional 'frontal' tasks correlate with both frontal lobe and parietal lobe function, and we suggest that pathological changes in the left parietal lobe may cause, in part, disturbances in executive tasks in PD.

Three-dimensional stereotactic surface projection study of orthostatic hypotension and brain perfusion image in Parkinson's disease.

OBJECTIVE: To compare brain perfusion image using three-dimensional stereotactic surface projection (3D-SSP) analysis of N-isopropyl-p-123I iodoamphetamine (123I-IMP) single photon emission computed tomography (SPECT) between patients with Parkinson's disease with orthostatic hypotension and those without orthostatic hypotension. MATERIALS AND METHODS: Fifteen patients with Parkinson's disease and orthostatic hypotension and 13 patients with Parkinson's disease without orthostatic hypotension were studied. We compared brain perfusion image between the two groups by 3D-SSP. RESULTS: Bilateral anterior cingulate gyrus perfusion of the patients with orthostatic hypotension was significantly decreased compared to that of the patients without orthostatic hypotension. CONCLUSIONS: The disorder of anterior cingulate gyrus may participate in the autonomic failure in Parkinson's disease.

Severe brain atrophy in a case of thalamic variant of sporadic CJD with plaque-like PrP deposition.

A 30-year-old woman presented with ataxic gait and progressive mental deterioration, and 3 years later developed myoclonus in the limbs. Subsequently, she lapsed into an akinetic state and died more than 6 years after the onset of disease. The brain weighed 670 g, and preferential degeneration was found in the medial thalamus and the inferior olivary nucleus. In the cerebrum and cerebellum, gliosis and neuronal depletion were only mild and disintegration of the parenchymal structures was inconspicuous, despite pronounced atrophy. The patient had methionine homozygosity at codon 129 of the PrP gene and protease-resistant PrP type 2 in the brain. On PrP immunostaining, plaque-like deposits were detected in the cerebral and cerebellar cortices. Severe brain atrophy such as in the present case has never been described in the thalamic variant of sporadic CJD.

[Cerebral infarction presenting pure motor monoparesis: diagnosis by diffusion-weighted MR imaging].

We studied 10 patients with acute ischemic cerebrovascular disorders presenting paralysis confined to one limb, unaccompanied by sensory signs(pure motor monoparesis, PMM) on diffusion-weighted MR imaging(DWI). DWI revealed fresh ischemic lesions in all patients, except for 2 cases of transient ischemic attack. On DWI, acute infarction in multiple lesions was identified, and small superficial lesions were clearly described. Superficial lesions were seen in 4 patients, and deep lesions were also seen in 4 patients. DWI is useful for lesion analysis in cerebral infarction with PMM.

Cerebral white matter lesions are not associated with apoE genotype but with age and female sex in Alzheimer's disease.

Cerebral white matter lesions, such as leukoaraiosis, may be a result of damage from cerebral ischaemia, and may also be associated with the degenerative process in Alzheimer's disease. The apolipoprotein epsilon4 (apoepsilon4) genotype is a genetic risk factor for both Alzheimer's disease and ischaemic brain damage through acceleration of atherosclerosis. The aim was to determine whether apoepsilon4 may be related to the formation of cerebral white matter lesions in Alzheimer's disease. The association of apoE genotype, sex, age, and the presence of several vascular risk factors, with the presence of white matter lesions in 55 patients clinically diagnosed with Alzheimer's disease was investigated. The cerebral white matter lesions were identified by T2 weighted MRI and classified on a 4 grade scale from no lesion to diffuse lesion. The odds ratio (OR) of the factors mentioned above to the presence of white matter lesions was determined and tested by Fisher's exact test. The association of the lesion grades with these factors was analysed by non-parametric tests. The apoE 4 genotype was strongly associated with Alzheimer's disease (p=0.0001), but not associated with the presence or the degree of cerebral white matter lesions in Alzheimer's disease (OR=1.09, p>0.99). Aging (>70 years old) was a significant risk factor for white matter lesions (OR=7.2, p=0.0006) and age was significantly correlated with the lesion (p=0.0075). The OR of female sex to the lesion grades was 2.89 (p=0.084) and the lesion grade of female sex was significantly higher than that of the male sex (p=0.047). Other vascular risk factors were not significantly associated with the presence of white matter lesions. These findings suggest that there is a sex difference in white matter pathology in Alzheimer's disease.

[A case of adult-onset neurenteric cyst presenting as chronic progressive muscular atrophy in lower legs].

A 50-year-old man was admitted, because of motor weakness of the lower limbs, dysesthesia of the left lower extremity, and anuresis. He had an episode of pain in his gluteal region 17 years ago, and then, no abnormalities were detected including myelography in a hospital, followed by slowly progressive muscular atrophy of his lower legs. At 50 years of age, dysuria appeared. He was diagnosed as having neurogenic bladder by urologists, and was admitted to our hospital. On admission, abnormal neurologic findings included: severe muscular atrophy in his lower legs, pes cavus, dysesthesia at the left S1 level, and autonomic bladder. Magnetic resonance imaging (MRI) showed mass lesion involving lower conus and cauda equina. After resection, pathological study revealed the mass was a neurenteric cyst. It is said that the neurenteric cyst causes an asymmetrical and sequential loss of specific neurological functions, with a subsequent return of these functions in the reverse order. That mechanism is not clear. However, in our case, the course of the illness was slowly progressive. We speculate that, because of the cyst's adhesion to cauda equina and perforation through the cyst by a nerve root, the cyst was fixed and caused slowly progressive neurological deficits in proportion to increase of the cyst's size. Our report suggests that a neurenteric cyst, involving the lower conus and cauda equina, can produce severe muscular atrophy in the lower legs.

[Secondary parkinsonism following midbrain hemorrhage].

We report a patient who developed right sided cogwheel rigidity and resting tremor after left midbrain hemorrhage. Brain magnetic resonance imaging (MRI) showed left midbrain old hemorrhage including substantia nigra. I-123 iodoamphetamine single photon emission computed tomography (IMP-SPECT) images showed reduced radioisotope (RI)-uptake in the left striatum, thalamus and frontal lobe. Our report shows that focal midbrain lesion can produce parkinsonism.

[Disappearance of essential neck tremor after pontine base infarction].

Mechanism of essential tremor remains unknown. Central oscillators, postulated in thalamus, inferior olive, and spinal cord are thought to be important to form rhythmicity, and finally to stimulate spinal or medullary motor cells, leading trembling muscle contraction, tremor. Among several subtypes of essential familial tremor, including hand tremor, neck tremor, and voice tremor, essential neck tremor is a common disorder, and its pathophysiology seems different from that of typical essential hand tremor, since patients with essential hand tremor are responsive to beta blocker, whereas those with neck tremor are usually not. We experienced a 41-year-old left handed woman with essential neck tremor in whom neck titubation disappeared shortly after pontine base infarct. She was our patient in the outpatient clinic with the diagnosis of essential neck tremor. The tremor developed when she was teenage, and has been localized in the neck muscles. Alcohol intake had apparently diminished it transiently. Her mother also had the tremor in her neck. She was admitted to our hospital with sudden onset of right-sided limb weakness and speech disturbance. Neurological examination showed right hemiparesis including the ipsilateral face, scanning speech, and cerebellar limb ataxia on the same side. In addition, there was no tremor in her neck. Brain MR imaging revealed a pontine base infarct at the level of middle pons, which was consistent with paramedian artery territory. The hemiparesis and speech disturbance improved almost completely after treatment, and her neck tremor has never occurred in one year follow-up. In our patient, efficacy of alcohol imply that essential neck tremor and hand tremor had same central nervous pathway including central oscillator in common, and descending cortical fibers is seemingly associated with diminishing patient's tremor. Pathophysiology of essential neck tremor was discussed with reviewing previous literature.

Anorexia nervosa with recurrent hypoglycemic coma and cerebral hemorrhage.

A 22-year-old woman with anorexia nervosa showed recurrent hypoglycemic coma and cerebral hemorrhage (Weber's syndrome). She also showed marked liver dysfunction. However, the liver damage was unlikely the main cause of these complications, because liver function tests were improved, and platelet count and prothrombin time were normal when cerebral bleeding occurred. These complications could be induced by her severe malnutrition. Although she rejected any nutritional support before cerebral hemorrhage, she began to accept glucose infusion after the episode, and psychiatric and nutritional states were apparently improved. Cerebral hemorrhage was a turning point in her disease.

MRI demonstration of a reversible lesion in cerebral deep white matter in thrombotic thrombocytopenic purpura.

We report a 75-year-old woman with thrombotic thrombocytopenic purpura (TTP) whose MRI showed diffuse abnormal signal in the deep white matter. She was successfully treated, and this abnormal signal disappeared. This finding indicates that the deep white matter is involved in TTP; the lesion may reflect reversible microangiopathy and brain oedema.

[A case of a thoracic extradural arachnoid cyst presenting with slowly progressive muscle weakness in the right upper and lower limbs].

A 49-year-old woman developed slowly progressive muscle weakness of the right upper and lower limbs. Physical examination revealed exaggeration of deep tendon reflexes in bilateral lower extremities and a Th5-Th6 girdle sensation. Weakness in her right upper extremity suggested cervical or intracranial lesion. Neuroradiological studies detected no abnormalities in her cervical cord and cranium. So the symptoms and signs were similar to those of motor neuron disease except for the sensory disturbance. MRI study of thoracic cord demonstrated a thoracic extradural arachnoid cyst. After removal of the cyst, the patient's muscle weakness was prominently relieved. We postulate that the cyst stretched spinal cord and dura mater, which led to affection of her cervical cord. We propose weakness of an upper limb as a pseudo-localizing sign of a thoracic extradural arachnoid cyst.

[Persistent cerebellar ataxia following typhoid fever].

We report a patient who developed acute cerebellar ataxia, delirium, disseminated intravascular coagulation (DIC), and acute renal failure following typhoid fever. After treatment his general conditions improved except for cerebellar ataxia. Brain magnetic resonance imaging (MRI), before and after intravenous gadolinium administration, showed slight atrophy of the cerebellar vermis. 1-123 iodoamphetamine single photon emission computed tomography (IMP-SPECT) images showed markedly reduced radioisotope (RI) uptake in the cerebellum, and strongly implicated dysfunction of the cerebellum. Our report suggests that severe typhoid fever can produce persistent cerebellar dysfunction.

[Oncogenic osteomalacic myopathy; a case report].

A 54-year-old man with oncogenic osteomalacic myopathy was reported. He presented with gradual onset of muscle weakness and painful cramp of the bilateral quadriceps femoris muscles, followed by low inorganic phosphorus, elevated alkaline phosphatase in the serum and hyperphosphaturia. The electromyogram (EMG) revealed myogenic change localized in the quadriceps muscle and nerve conduction study was normal. Muscle biopsy of right vastus lateralis muscle demonstrated non-specific myopathic change with minimal neuropathic change: moderate variation in size, many centrally placed nuclei, a few small angulated fibers and pyknotic nuclear clump. The ratio of Type IIA fibers decreased to 5.5%. Serum 25-hydroxyvitamin D and parathormone were normal, whereas 1, 25-dihydroxyvitamin D was above lower normal limit. High dose of 1,25-dihydroxyvitamin D and phosphorus were administered with partial response. CT scan demonstrated tumor in the left 10th rib proximal to the vertebra, invading into the mediastinum. Partial resection of the tumor was performed in order to preserve the rib. Histologically, the tumor was characterized by high vascularity with cystic formation, many giant cells and tumor cells with oval-shaped nuclei. Histopathological diagnosis was primitive mesenchymal tumor (mixed connective tissue variant). Immediately after resecting the tumor, the patient's muscle weakness and painful cramp was prominently relieved and serum inorganic phosphorus and tubular reabsorption of phosphorus became normal, in addition, 1, 25-dihydroxyvitamin D was elevated within normal limit. EMG findings revealed normal motor nerve unit with normal recruitment pattern. Mild myopathy is relatively a common manifestation in patients with osteomalacia. On the other hand, osteomalacia is sometimes caused by tumors, many of which are benign mesenchymal tumors of bone or soft tissue origin.(ABSTRACT TRUNCATED AT 250 WORDS)

SPECT findings in Parkinson's disease associated with dementia.

Dementia in Parkinson's disease is thought to be attributable not only to subcortical lesions but also to cortical alterations, especially frontal lobe dysfunction. To evaluate cortical function, the regional cerebral blood flow (rCBF) was estimated of 13 demented and 13 non-demented age matched patients with Parkinson's disease compared with that of 10 age matched controls using I-123 iodoamphetamine single photon emission tomography (IMP-SPECT). The rCBF of the nondemented Parkinson's patients showed no significant differences from that of the control subjects. In the demented patients, the bilateral frontal and parietal and left temporal regional blood flow was significantly less than in the controls. Four demented patients showed isolated frontal hypoperfusion, 8 showed fronto-parietal hypoperfusion, and 1 showed isolated parietal hypoperfusion. Frontal hypoperfusion was therefore present in 12 of the 13 demented patients, and this finding agrees with the frontal lobe dysfunction hypothesis. Parietal rCBF had a significant positive correlation with cortical functions such as calculation and language ability in the MMSE scores. The parietal and temporal reduction in rCBF probably reflects the presence of Alzheimer pathology, cortical Lewy body disease, or both.

Changes in extracellular matrix materials in the uterine myometrium of rats during pregnancy and postparturition.

Morphological changes in extracellular matrix materials in the uterine myometrium of rats during pregnancy and postparturition were studied by light and electron microscopy together with immunofluorescence microscopy for type III and IV collagens, fibronectin and laminin. The main components present in late pregnancy were 1) various-sized collagen fibrils, 2) thick elastic fibers adjacent to smooth muscle cells, and 3) continuous and thick basement membranes of hypertrophic smooth muscle cells. These findings are considered to indicate degradation of collagen fibrils and development of elastic fibers and basement membranes of smooth muscle cells. This change in extracellular matrix materials in the late stage of pregnancy may be important in the process of uterine enlargement associated with elasticity and preparation for labor. In the postpartum stage, myofibroblastic interstitial cells were seen to phagocytize collagen fibrils, and elastic fibers accumulated mainly around the bundles of smooth muscle cells. These changes in the postpartum stage are thought to be important for the process in which the uterus returns to the nonpregnant condition. It is suggested that smooth muscle cells participate in regulating the development of their basement membranes and elastic fibers, and that myofibroblastic interstitial cells function by clearing degraded collagen fibrils from the uterine myometrium.