Fractal dimension analysis of static stabilometry in Parkinson's disease and spinocerebellar ataxia.

The static stabilometry patterns associated with Parkinson's disease (PD, n = 15) and spinocerebellar ataxia (SCA, n = 15) were compared with those of normal control (n = 15) by measuring the fractal dimensions. Fractal dimensions were estimated using the modified pixel dilation (mPD) method. The fractal dimensions with closed eyes showed a significant correlation with Environmental area for SCA group (p < 0.05). The fractal dimension for SCA group was significantly higher with closed eyes than that with open eyes (p < 0.05). The fractal dimension with closed eyes was significantly higher in PD and SCA groups than that in normal group (p < 0.05). The fractal dimension with closed eyes was higher when the clinical stage was more severe with PD and SCA group while Environmental and Longitude/Environmental areas were not. These findings suggest that the fractal dimension is more sensitive than traditional stabilometric analysis in an evaluation of postural instability in PD and SCA.

[A case of spinocerebellar ataxia type 6 with hypochondriasis and severe parkinsonism].

We report a case of 68-year-old woman who was diagnosed spinocerebellar ataxia type 6 (SCA 6) by genomic testing. She presented hypochondriasis, parkinsonism, and ataxia. Since the age of 60, she noted difficulty in walking due to dizziness, and MRI showed minimal cerebellar atrophy. She became unable to walk without assistance at the age 67. She was referred to us when she was 68 years old. She had no family history of cerebellar ataxia, and her general physical examination was normal. Her speech was fluent, with neither slurring nor scanning, and she complained of much anxiety regarding her physical condition and was diagnosed as having hypochondriasis. Neurological examination revealed parkinsonism consisting of small steppage gait, mask-like face, akinesia, rigidity of neck and limbs, and postural instability. She also showed cerebellar signs such as saccadic smooth pursuit, ataxia of upper and lower limbs, and increased tendon reflexes. Her parkinsonism had developed slowly and symmetrically yet she showed a lack of response to levodopa. Our results suggest that the genomic testing is useful for differential diagnosis for the diseases presenting ataxia and parkinsonism, even if the family history is negative.

Elevation of neuron-specific enolase in serum and cerebrospinal fluid of early stage Creutzfeldt-Jakob disease.

OBJECTIVE: To investigate the levels of neuron-specific enolase (NSE) in serum and cerebrospinal fluid (CSF) of patients with early stage Creutzfeldt-Jakob disease (CJD). METHODS: The levels of NSE in serum and CSF were examined in 6 cases with CJD patients. The levels of NSE in CSF were measured in 8 age matched control patients with other neurological diseases and the levels of serum NSE were also measured in another 8 age matched control patients with other neurological diseases. The groups of 8 age matched control patients consisted of 1 same patient and 7 different patients in the 2 control groups both for serum and CSF. RESULTS: The level of serum NSE in CJD (17.3 +/- 7.0 ng/ml, mean +/- SD) was significantly higher than that of controls (6.5 +/- 1.6) (P < 0.02) as was the case in CSF (79.3 +/- 53.3 ng/ml) vs (9.6 +/- 2.9) (P < 0.03). CONCLUSION: Although mean NSE levels of CJD were higher in CSF than in the serum, there still is a case with higher serum NSE level than CSF. These results suggest that the mechanism of elevation of serum NSE may not be a simple leakage from CSF, and that the measurement of serum NSE level may be useful for diagnosis of early stage CJD.

Temporal changes in expression of neuronal nitric oxide synthase mRNA in the rat hippocampus associated with kainate-induced seizures.

We studied the temporal changes in expression of neuronal nitric oxide (NO) synthase (nNOS) mRNA in the hippocampus of rats treated with kainic acid by use of in situ hybridization technique. Intraperitoneal injection of 10 mg kg-1 kainic acid decreased expression of nNOS mRNAs in the dentate gyrus and CA3 region of the hippocampus at 3 h and 8 h and increased it in the dentate gyrus and CA1 at one week after treatment. Although our previous study indicated that administration of kainic acid increased NO generation in the rat hippocampus, present results suggest that the injection of kainic acid results in differential regulation of nNOS mRNA and NO formation in the rat hippocampus.

Laminar cortical necrosis in central nervous system lupus: sequential changes in MR images.

A 44-year-old woman with systemic lupus erythematosus developed central nervous system lupus presenting with headache, fever, cloudiness of consciousness, and psychotic symptoms. T1-weighted and proton MR images showed laminar high intensity lesions in the parietal and temporal cortex bilaterally. T2-weighted images of the lesions showed low signal intensity. Treatment with corticosteroids alleviated the clinical symptoms within 7 months. The low T2 and high T1 signal abnormalities disappeared in a year and in 5 years, respectively. The laminar cortical lesions on MR images were suggested to represent cortical necrosis associated with central nervous system lupus.

Hyperintense basal ganglia on T1-weighted MR images in a patient with central nervous system lupus and chorea.

We describe a patient with central nervous system lupus and choreatic movements in whom both basal ganglia showed high signal intensity on T1-weighted MR images, while the signal on T2-weighted images remained low. Within 8 months after onset, the choreatic movements had disappeared, with a corresponding decrease in the hyperintense T1 signal. The emergence of the choreatic movement disorder in this patient might have been related to the T1 hyperintensity of the basal ganglia, which, in turn, might have resulted from a vascular insult associated with central nervous system lupus.

[The use of magnetic resonance imaging (MRI) and single photon emission computing tomography (SPECT) for the differential diagnosis of Parkinson's disease and other neurodegenerative disorders presenting as parkinsonism].

In Parkinson's disease, both MRI and SPECT are usually normal. In striatonigral degeneration and olivo-ponto-cerebellar atrophy presenting as parkinsonism, MRI shows putaminal atrophy and SPECT shows hypoperfusion in the frontal lobe, basal ganglia and cerebellum. In progressive supranuclear palsy, MRI shows tegmental atrophy and SPECT shows hypoperfusion in the frontal lobe with intact cerebellar perfusion. In conclusion, MRI and SPECT seem to be useful for the differential diagnosis of Parkinson's disease and other neurodegenerative disorders presenting as parkinsonism.

Long-lasting increase in protein kinase C activity in the hippocampus of amygdala-kindled rat.

Previous studies have demonstrated that membrane-associated protein kinase C (PKC) activities in the right and left hippocampus of rats kindled from the left hippocampus increased significantly at 4 weeks [9] and 4 months [22] after the last seizure compared with those in matched control rats. In this study, we investigated the effect of kindling from the left amygdala on PKC activities in the amygdala/pyriform cortex and hippocampus at long seizure-free intervals (4 and 16 weeks) from the last amygdala-kindled seizure. Membrane-associated PKC activity of the kindled group increased significantly only in the left hippocampus compared with the left side control (the left hippocampus of rats subjected to a sham operation) at 4 weeks (by 34%, P < 0.03) and 16 weeks (by 24%, P < 0.05) after the last seizure. There was no significant alteration in the membrane-associated PKC activity of the kindled group in the right hippocampus or amygdala/pyriform cortex in any seizure-free interval after the last amygdala seizure. Cytosolic PKC activity did not differ between the kindled and control groups in any brain region examined in any seizure-free interval. At 16 weeks after the last seizure, the PKC activity in the P1 fraction of the kindled group increased significantly only in the left hippocampus (by 49%, P < 0.005), but not in the right hippocampus. Neither PKC activity in the P2 fraction nor that in the cytosolic fraction was altered in the kindled group after this seizure-free interval.(ABSTRACT TRUNCATED AT 250 WORDS)

Hepatic encephalopathy with increased cerebral blood flow in SPECT and MRI abnormalities in the basal ganglia.

A case of hepatic encephalopathy with increased cerebral blood flow in single-photon emission computed tomography (SPECT) and abnormalities upon magnetic resonance imaging (MRI) is reported. SPECT showed increased blood flow exceeding 70 ml/100 g/min in the cerebellum, basal ganglia and cerebral cortex, with hyperammonemia and abnormal electroencephalographic signs showing severe generalized slowing. SPECT data and MRI might reflect abnormal metabolism and neuropathological changes in hepatic encephalopathy.

MELAS without ragged red fibers or lactic acidosis diagnosed by mitochondrial DNA testing.

A case of mitochondrial encephalomyopathy with lactic acidosis, a stroke-like episode (MELAS) without ragged red fiber, diagnosed by mitochondrial DNA testing, is reported. A 37-year-old woman experienced a sudden and recurrent headache with vomiting and stroke-like episodes. Brain CT and MRI showed multiple infarction in the temporal lobes, not corresponding to artery distribution. However, the plasma levels of lactate and pyruvate were normal, and showed no increased after aerobic exercise. Biopsied muscle showed no evidence of ragged red fibers and deficient activity of mitochondrial enzymes in the respiratory chain. The final diagnosis was made by mitochondrial DNA testing. A southern blot analysis after Apa I digestion revealed the A-to-G mutation in the tRNA(Leu(UUR)), which is specific to MELAS.

Pure sensory seizures.

A 62-year-old woman, who frequently had numbness seizures in the right half of her body, is reported. Weakness and consciousness disturbance did not occur during the seizure, and an abnormal electroencephalogram was not observed during the seizure or between seizures. The seizure was completely inhibited by a single-blind administration of phenytoin. It also disappeared on administration of carbamazepine, suggesting that the numbness seizures in this patient were of an epileptic nature.

[High-field-strength magnetic resonance imaging and SPECT of multiple system atrophy].

Two cases of multiple system atrophy (MSA) showing similar abnormalities by magnetic resonance (MR) imaging and SPECT are reported. The clinical diagnoses of the two cases were striatonigral degeneration (SND) and sporadic olivopontocerebellar atrophy (OPCA). In addition, one case of sporadic OPCA without parkinsonism was used for comparison. The MR images were obtained using a 1.5-T MR system and included spin-echo transverse sections with T1-weighted images (TR = 450 ms and TE = 15 ms) and T2-weighted images (TR = 2500 ms and TE = 90 ms). The T1-weighted images demonstrated atrophy of cerebellum and pons, with increased signal intensity in the bilateral putamen. The T2-weighted images demonstrated decreased signal intensity in the putamen, as reported recently. SPECT demonstrated reduced uptake in the celleberum, basal ganglia and frontal lobe cortex. The putaminal changes evident on T1-weighted images may have resulted from deposition of pigments such as neuromelanin and lipofuscin, related to parkinsonism. Both T1- and T2-weighted MRI seem to be useful clinical diagnosis of MSA.

Enduring increase in membrane-associated protein kinase C activity in the hippocampal-kindled rat.

In a previous study we demonstrated that the membrane-associated protein kinase C (PKC) activity in the amygdala/pyriform cortex (AM/PC) and both the right and left hippocampus (HIPP) of rats kindled from the left HIPP increased significantly 4 weeks after the occurrence of the last seizure compared with control rats. In this study, we carried out further investigations into the enduring effect of HIPP-kindling on membrane-associated PKC activity, the protein concentrations and brain wet tissue weights of the AM/PC and right (contralateral) and left (ipsilateral) HIPP 15-16 weeks after the last generalized kindled seizure had occurred. In addition, we determined the membrane-associated PKC activity one week after the occurrence of the last partial (stage 1-3) seizure. Fifteen to sixteen weeks after the final kindled full seizure, the membrane-associated PKC activity which was expressed as mol/min per mg protein increased significantly in the AM/PC (by 62%, P < 0.02) and left HIPP (by 33%, P < 0.03) compared with control rats, whereas the cytosolic PKC activities did not differ in any brain region examined. The wet tissue weight increased significantly (by 10%, P < 0.04) in the left HIPP only. Furthermore, when it was expressed as pmol/min per mg wet tissue weight, the membrane-associated PKC activity increased significantly in the AM/PC (by 47%, P < 0.02), right HIPP (by 27%, P < 0.05) and left HIPP (by 35%, P < 0.03) compared with the controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Ionotropic excitatory amino acid receptors in discrete brain regions of kindled rats.

A study was performed to examine the specific binding of excitatory amino acid (EAA) receptor subtypes in 5 brain regions of rats kindled from the amygdala or hippocampus, using extensively washed and Triton X-100-treated membranes. Seven days after the last amygdala kindled seizure, [3H](+)-5-methyl-10,11-dihydro-5H-dibenzo[a,d]cyclohepten-5,10- imine maleate ([3H]MK-801) binding, which labels N-methyl-D-aspartate (NMDA)-sensitive receptor-linked cation channels, decreased significantly only in the amygdala of kindled rats compared to that of controls under equilibrium assay conditions. There was no significant change in [3H]MK-801 binding in the amygdala or hippocampus 7 days after the last hippocampal kindled seizure, or 28 days after the last amygdala kindled seizure. Nor was there a significant change in NMDA-sensitive [3H]glutamate, strychnine-insensitive [3H]glycine, [3H]spermidine, [3H]kainate or [3H]alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid ([3H]AMPA) binding in any brain region 7 days after the last amygdala kindled seizure, or in the hippocampus 28 days after the last amygdala kindled seizure. These results indicate that [3H]MK-801 binding sites labeling NMDA-sensitive receptor-linked cation channels in the amygdala undergo downregulation only transiently, but that none of the subcomponents of the NMDA receptor macromolecular complex exhibit enduring changes at steady state following the completion of amygdala kindling.

[A case of encephalitis with MRI abnormalities localized in the bilateral amygdala and hippocampus].

We report a patient with encephalitis who showed anterograde and retrograde amnesia with MRI abnormalities localized in the bilateral amygdala (AM) and hippocampus (HIPP). A 25-year-old man suddenly experienced a generalized tonic-clonic seizure (GTCS). He was admitted because of increasing lethargy with two further GTCSs during the following 6 days. The patient had high fever, and neurological examination revealed somnolence, disorientation, amnesia, and nuchal stiffness. MRI revealed bilateral symmetrical abnormalities localized in the AM and HIPP, which showed low intensity on T1-weighted images and high intensity on T2-weighted images. Cerebrospinal fluid examination showed a mildly elevated cell count. We suspected herpes simplex virus type I encephalitis and began treatment with acyclovir. After the patient regained a clear consciousness, his antero- and retrograde amnesia continued for several months. The MRI abnormality became less distinct with the improvement of amnesia. We consider that the MRI abnormality was indicative of inflammation and edema, and that the lesion in the AM and HIPP had induced the amnesia.

[Diffuse reduced uptake in 99m-Tc-HM-PAO SPECT and high level of neuron-specific enolase in cerebrospinal fluid in the early stage of Creutzfeldt-Jakob disease].

We report an autopsy-diagnosed case of Creutzfeldt-Jacob disease (CJD) showing diffuse reduced uptake in SPECT and a high level of neuron-specific enolase (NSE) in the cerebrospinal fluid (CSF). A 75-year-old woman with a 3-month history of progressive gait ataxia and dementia was examined. Four months after onset of the disease, she developed an akinetic mutism with normal CT and MRI. She was admitted for further examination, and electroencephalography showed periodic synchronous discharges. NSE in the CSF showed a high level at 90.6 ng/ml, and SPECT showed diffuse reduced uptake in the supratentorial region, with relatively spared uptake in the cerebellum. She died 13 months after onset of the disease, and the autopsy diagnosis was CJD. SPECT data and the level of NSE in CSF seem to be useful for clinical diagnosis and pathogenetic analysis in the early stage of CJD.

[A case of SSPE presenting visual disturbance and serial MRI study].

A case of subacute sclerosing panencephalitis (SSPE) was reported. The patient was a 16-year-old boy and he initially developed visual disturbance. His neurologic symptoms were myoclonus, dementia, and visual disturbance which was rare as an initial symptom in SSPE. Fundoscopy revealed bilateral pole chorioretinitis and macular degeneration. Serial MRI study demonstrated the lesions in the brain and right eye ball. The distribution varied as time went on. T2-weighted MR images showed the lesions more clearly than T1-weighted MR images. In this case dementia was marked but the lesions in the cerebrum on the MR images were considerably smaller than expected. Although MR image is useful to show the lesions, the discrepancy between clinical signs and MR images may be present in the early stage in SSPE.

[CSF monoamine metabolites in patients with progressive supranuclear palsy].

Neurochemical abnormalities in the patients with progressive supranuclear palsy (PSP) are not well understood. We investigated CSF levels of the monoamine metabolites HVA, 5HIAA and MHPG in 5 patients with PSP in order to investigate, especially dopaminergic, serotonergic and noradrenergic metabolism. Results were as follows (value; mena +/- S.D.). [table: see text] The levels of HVA were significantly lower than those in controls. These findings suggest that there is a hypofunction of dopaminergic neurons in PSP patients.