Ariyuki Hori

Paramedian Pontine Infarction: Neurological/Topographical Correlation

BACKGROUND AND PURPOSE There have been few reports of pontine syndromes secondary to paramedian pontine infarctions. To clarify the clinicotopographical correlation and prognosis of paramedian pontine infarct syndromes, we analyzed the clinical signs and their association with MRI findings. METHODS We studied 49 patients with acute paramedian pontine infarcts and classified them into three subtypes on the basis of lesion location on MRI. Patient clinical status was assessed by Rankin Disability Scale (RDS) scores on admission and at 60 days after onset of stroke. RESULTS Twenty-seven patients had basal infarcts. Clinical findings included dysarthria (n = 27), hemiparesis with upper extremity predominance (n = 15), brachial monoparesis (n = 4), and pathological laughing (n = 3). Fifteen patients had basal-tegmental infarcts. Clinical findings presented with hemiparesis and horizontal gaze abnormalities, including abducens nerve palsy (n = 1), internuclear ophthalmoplegia (INO) (n = 5), horizontal gaze palsy (n = 1), one-and-a-half syndrome (n = 1), and superficial or proprioceptive sensory dysfunction (n = 8). Seven patients had tegmental infarcts. Clinical findings included INO (n = 1), horizontal gaze palsy (n = 2), one-and-a-half syndrome (n = 3), and sensory changes (n = 2). On both admission and 60 days later, the RDS scores of the patients with upper pontine lesions were significantly better than those with lower pontine lesions (P < .01). The RDS scores of the patients with basal-tegmental infarct in the upper pons were significantly better than those with infarct in the lower pons (P < .02). CONCLUSIONS Paramedian pontine infarcts, which are usually due to thrombosis of perforating arteries, presented with a faciobrachial dominant hemiparesis with dysarthria, somatosensory disturbance, and horizontal gaze abnormalities. The favorable outcome may be related to the level of the pontine lesion, which influences the effect on the corticospinal tract.

Cardiac 123I-MIBG scintigraphy can assess the disease severity and phenotype of PD

OBJECTIVE Cardiac (123)I-metaiodobenzylguanidine (MIBG) scintigraphy studies of patients with idiopathic Parkinsons disease (PD) found decreased uptake. Whether this decrease is associated with clinical severity as assessed by the Unified Parkinsons Disease Rating Scale (UPDRS) and the phenotypes of PD has not been determined. METHODS Cardiac MIBG scintigraphy was performed on 34 patients with PD, 7 with multiple system atrophy (MSA), 4 with dementia with Lewy bodies (DLB), and 11 normal controls (NCs). Early and delayed MIBG heart/mediastinum (H/M) ratios were evaluated. PD severity was assessed by the Hoehn and Yahr (H-Y) stage and UPDRS. Patients were grouped in two phenotypes, tremor and postural instability gait difficulty (PIGD)-dominant groups based on UPDRS components. Associations between MIBG uptake and age at onset, UPDRS, and disease phenotype were analyzed in each group. RESULTS The early H/M ratio was significantly lower in patients with PD (1.45+/-0.207) than in the NCs (2.08+/-0.231), and in those with MSA (1.99+/-0.284), but not in those with DLB (1.29+/-0.0435). The delayed H/M ratio for PD (1.33+/-0.276) also was significantly decreased as compared to the ratios for NCs (2.17+/-0.286) and MSA (2.16+/-0.414) but not DLB (1.16+/-0.0949). The early H/M ratio was significantly correlated with both UPDRS score and age at onset, whereas the delayed H/M ratio only was significantly correlated with age at onset. The PIGD-dominant group had significantly higher UPDRS scores and lower H/M ratios than the tremor-dominant group. CONCLUSION Cardiac MIBG scintigraphy can be used to differentiate PD from MSA and NC, and to determine the disease severity and phenotypes of PD.

CHANGES IN CEREBRAL BLOOD FLOW VELOCITY IN HEALTHY YOUNG MEN DURING OVERNIGHT SLEEP AND WHILE AWAKE

Using transcranial Doppler ultrasonography, we measured in 6 healthy young men cerebral blood flow velocity (CBFV) in the middle cerebral artery as well as oxygen saturation by a non-invasive method. Continuous recordings were taken starting from a point before the onset of sleep, throughout the duration of normal nighttime sleep, ending after awakening. During stages 2, 3 and 4, CBFV was approximately 15% lower than during the waking period preceding sleep. CBFV during rapid eye movement sleep did not differ from the presleep waking value, whereas the postsleep waking value was 6.6% lower. In 5 subjects CBFV showed a transient rise after sleep onset. Oxygen saturation was lower during sleep than during waking.

Sleep characteristics in twins.

Abstract: Polysomnograms were recorded for three consecutive nights on 14 male students (mean age, 16), comprising 4 identical and 3 fraternal twin pairs. The number of body movements and the measures related to the REM cycle, which correlated among the identical twins without reference to the physical parameters, were considered to be determined by a genetic trait. The sleep spindle density was in almost complete concordance between the identicaltwins and was associated with some physical parameters, which suggests that sleep spindles rather reflect the individual development. %S3, %SREM and the number of SREM showed a relationship to the physical parameters without heritability. Twitch movements during REMsleep correlated not only between the identical twins but also between the fraternal twins.

Chorea-acanthocytosis associated with Tourettism.

We report on a case of Chorea‐acanthocytosis (ChAc) in association with Tourettism that consisted of motor and vocal tics, attention deficit–hyperactivity disorder, and obsessive–compulsive disorder in addition to the typical symptoms of ChAc. The subject was compared with his elder sister who had the same disease but milder clinical profile and neuroradiological findings. The [18F]‐2‐fluoro‐2‐deoxyglucose positron emission tomography (FDG‐PET) findings did not explain the differences in symptomatology between the patient and his sister, although they may have correlated with severity.

Activation of thrombosis and fibrinolysis following brain infarction

To clarify the sequence of alterations in the thrombotic and fibrinolytic systems after acute brain infarction, we prospectively examined sequential changes in coagulatory markers in 38 patients suffering from cardioembolic infarcts (CEI), 41 patients with atherothrombotic infarcts (ATI), 58 patients with lacunar infarcts (LI), and 32 age-matched controls. The plasma level of thrombin-antithrombin III complex (TAT), fibrinopeptide A (FpA), D-dimer, fibrin degradation products-E (FDP-E), fibrinogen, alpha2-plasmin inhibitor-plasmin complex (PIC), and percent activity of antithrombin III (AT-III) were measured within 48 h, at 1 week, and at 3 weeks after the stroke onset. Significantly elevated levels of TAT and FpA, which are both markers of thrombin formation, were observed in CEI patients, and these elevated levels were associated with increasing D-dimer levels for 3 weeks (P<0.0001). D-Dimer in CEI patients was significantly elevated compared to control, LI and ATI levels within 48 h (P<0.001). Percent activity of AT-III was significantly decreased in CEI patients for 3 weeks compared to this activity in controls, LI and ATI (P<0.001). TAT and FpA also increased significantly within 48 h in ATI subjects and declined thereafter. A significant elevation of FDP-E (P<0.001) and D-dimer (P<0.05, P<0.01) was detected in parallel with increasing fibrinogen for 3 weeks. However, there was no significant depletion of percent activity of AT-III in ATI. In LI subjects, no significant elevation of TAT, D-dimer or FDP-E were observed within 1 week. PIC increased significantly in three subtypes of brain infarcts, but did not differ significantly among the three subtypes for 3 weeks. An accurate assessment of sequential alterations in thrombotic and fibrinolytic markers in the acute stage of brain infarct should contribute to the clinical diagnosis of brain infarct subtype. Alterations in these markers in response to activation of the coagulatory system are attributable to the different pathogenesis of ischemic stroke.

Twin Studies on Parasomnias

The parasomnias are classified as disorders of arousal, partial arousal, and sleep stage transition. Many of these parasomnias manifest dramatic symptoms causing an activation of central nervous system. Most parasomnias appear early in childhood. Some parasomnias, for example REM sleep behavior disorders (RBD) and sleep-related penile problems are seen in adult and aged people (Figure 1). Usually, these patients have predisposing factors including neurologic disorders and aging (Figure 2). Despite these explosive features, most of children with these parasomnias show spontaneous remission at the time of puberty.

Sleep Spindles in Twins

Sleep spindle characteristics and spindle power periodicity were studied in 4 identical and 3 fraternal twin pairs (mean age, 16 years). There were no significant genetic effects such as concordance between the identical twins and discordance between the fraternal twins for mean duration, mean amplitude and mean frequency of spindles. Spindle periodicity, which is correspondent to the sleep cycle, was visually more similar between the identical twins than between the fraternal twins. These observations suggest that the sleep cycle which is expressed by periodic appearance of spindle powers is genetically determined. On the other hand, some spindle characteristics and some physical measures had significant relationships. These relationships may suggest that some spindle characteristics are influenced by the individual development rather than by a genetic trait.

Electroencephalographic findings in neuroacanthocytosis

This study reports the electroencephalographic findings in two siblings with neuroacanthocytosis. Slowing of the background activity with diffuse slow waves was the main finding in the daytime EEG. All-night polysomnographic recordings were characterized by high voltage slow activity during drowsy state and REM sleep. One patient with an average of 40 episodes of apnea per night of sleep was treated with imipramine and showed respiratory improvement.

An inherited disorder characterized by repeated episodes of bilateral ballism: A case report

This case report describes two siblings with a dyskinetic form of cerebral palsy who had repeated episodes of fever‐induced bilateral ballistic movements. The boy and his sister experienced the first episodes during early childhood. The movements developed over several hours and required rapid intervention. Electroencephalograms during the attacks showed no paroxysms, and brain magnetic resonance imaging scans revealed no lesions. The brother died of acute renal failure at age 5 due to rhabdomyolysis after his fifth episode of prolonged bilateral ballistic movements. This is the first report of an inherited disorder characterized by repeated episodes of violent movements.