Weaning from long-term mechanical ventilation utilizing closed-loop ventilation mode (IntelliVent®-ASV®) in a patient with spinal cord injury.

INTRODUCTION: Cervical spinal cord injury with the C3 neurological level may cause respiratory failure and require long-term mechanical ventilation. Conventional weaning of spontaneous breathing trials is difficult to perform outside of intensive care or spinal cord units. CASE PRESENTATION: An 80-year-old man presented with total tetraplegia and restrictive respiratory failure that required assisted ventilation after a falling accident. Cervical spine magnetic resonance imaging showed cervical cord compression that was worst at the C3-C4 intervertebral level. He experienced unexpected cardiac arrest during the conventional weaning process of trials of intermittent spontaneous breathing in the intensive care unit. The automated weaning protocol utilizing a closed-loop ventilation mode (IntelliVent®-ASV ® ) was introduced 131 days after injury in our ward for chronically ill patients. The patient was successfully weaned 39 days after the introduction of the weaning protocol. DISCUSSION: An automated weaning protocol utilizing a closed-loop ventilation mode could be an optional procedure in patients with cervical cord injury on long-term mechanical ventilation, even in a ward for chronically ill patients where sufficient staff is not available. The efficacy and safety, and the cost-effectiveness of the procedure should be examined in larger spinal cord units.

Differences of Longitudinal Chromatic Aberration (LCA) between Eyes with Intraocular Lenses from Different Manufacturers.

Several researchers have studied the longitudinal chromatic aberration (LCA) of eyes implanted with an intraocular lens (IOL). We investigated the LCA of eyes implanted with yellow-colored IOLs from three different manufacturers: Alcon Inc., HOYA Corp., and AMO Inc. The number of subjects was 11, 16, and 16, respectively. The LCA of eyes implanted with SN60WF and SN60AT (Alcon Inc.), and with XY-1 (HOYA Corp.), was the same as that of phakic eyes. The LCA of eyes with ZCB00V (AMO Inc.) was smaller than that of phakic eyes. The LCA of eyes implanted with Alcon's and HOYA's IOLs, but not the LCA of eyes implanted with AMO's IOLs, was positively correlated with the powers of the IOLs. We also performed simulations to verify the impacts of LCA on visual performance for 4-mm pupil diameter; the simulations were a polychromatic modulation transfer function (MTF) and a visual Strehl ratio computed on the basis of an optical transfer function (VSOTF). We concluded that the differences between the LCA of different manufacturers do not affect visual performances when some extent of higher-order aberration (HOA) exists. The smaller HOA of AMO IOLs may enhance visual performance.

Verification of the lack of correlation between age and longitudinal chromatic aberrations of the human eye from the visible to the infrared.

Several researchers studied the longitudinal chromatic aberration (LCA) of the human eye and observed that it does not change due to age. We measured the LCA of 45 subjects' normal right eyes at three distinct wavelengths (561, 690, and 840 nm) using a Hartmann-Shack wavefront aberrometer (HSWA) while consecutively switching between three light sources for wavefront sensing. We confirmed that the LCA of the human eye does not change due to age between 22 and 57 years.

[Orbital apex syndrome without MRI lesion caused by ANCA-associated vasculitis].

A 73-year-old man developed double vision and a progressive loss of visual acuity of the left eye over one week. Examination showed disturbances of the left II, III, IV, and VI cranial nerves, that is, an orbital apex syndrome. A brain MRI showed abnormal T2-high signals in the right maxillary sinus and the left mastoid cells without abnormalities in the left orbital apex and the surroundings. Laboratory examination showed an elevated erythrocyte sedimentation rate and a positive perinuclear anti-neutrophil cytoplasmic antibody (MPO-ANCA). After two courses of methyl-prednisolone pulse treatment, his external ophthalmoplegia fully recovered and he regained his left eye's sight. MPO-ANCA was negative and MRI abnormalities were disappeared after treatment. Two years later, the patient developed upper respiratory symptoms associated with an elevation of MPO-ANCA titer, and rapidly progressive renal failure. Renal biopsy specimen showed fibrinoid necrosis with periarteriolar neutrophil infiltration, which suggested that the patient suffered from ANCA-associated vasculitis probably of Wegener's granulomatosis or microscopic polyarteritis. ANCA-associated vasculitis may present with a focal neurological syndrome such as the orbital apex syndrome without a lesion detectable with MRI.

Isolated ACTH deficiency presenting with a glucocorticoid-responsive triphasic wave coma.

We report the case of a 58-year-old woman who presented with acutely developed coma characterized by electroencephalographic triphasic waves (TWs) in the absence of metabolic derangement. The patient's coma and TWs were promptly resolved after the administration of glucocorticoids, and thereafter isolated ACTH deficiency was diagnosed. Isolated ACTH deficiency may present with glucocorticoid-responsive acute encephalopathy without hypoglycemia, hyponatremia, or systemic hypotension. Electroencephalographic TWs or bursts of slow waves may be a clue to the diagnosis of this rare condition in patients with coma of unknown origin.

[Tuberculous cranial pachymeningitis presenting with long-standing diffuse brain dysfunction].

We report a 59-year-old immunocompetent man presenting with slowly progressive gait unsteadiness, dysarthria, and clumsiness in writing over 6 months. There were bilateral pyramidal signs, pseudobulbar palsy, and attention deficits. Cerebrospinal fluid examination showed mild mononuclear pleocytosis, and magnetic resonance imaging revealed pachymeningeal pattern of contrast enhancement beneath the calvarium and the posterior cranial fossa. Interferon-gamma release assay in whole blood after stimulation by specific tuberculosis antigens was positive and repeat polymerase chain reaction assay detected Mycobacterium tuberculosis genome in the cerebrospinal fluid. After combination therapy with anti-tuberculous agents and corticosteroids, the patient's pachymeningitis regressed. Tuberculous cranial pachymeningitis may present with chronic diffuse brain dysfunction without headache, fever, or cranial nerve dysfunction.

[Descending control of quiet standing and walking: a plausible neurophysiological basis of falls in elderly people].

Quiet standing and walking are generally considered to be an automatic process regulated by sensory feedback. In our report "Astasia without abasia due to peripheral neuropathy," which was published in 1994, we proposed that forced stepping in patients lacking the ankle torque is a compensatory motor control in order to maintain an upright posture. A statistical-biomechanics approach to the human postural control system has revealed open-loop (descending) control as well as closed-loop (feedback) control in quiet standing, and fractal dynamics in stride-to-stride fluctuations of walking. The descending control system of bipedal upright posture and gait may have a functional link to cognitive domains. Increasing dependence on the descending control system with aging may play a role in falls in elderly people.

Hypertrophic pachymeningitis in an immunocompetent adult with positive Aspergillus DNA in the cerebrospinal fluid.

A 42-year-old immunocompetent man presented with subacute onset unilateral headache and associated lower cranial nerve palsies. Cranial magnetic resonance imaging showed enhancing thickened tentorium cerebelli and subtentorial dura mater. Cerebrospinal fluid examination revealed lymphocytic pleocytosis and positive polymerase chain reaction assay of Aspergillus DNA. While on voriconazole treatment a progressive increase was noted in subtentorial pachymeningeal hypertrophy, which was excised because of critical compression of the medulla. The excision material showed extensive fibrosis, cellular infiltrates and no organisms. With combination therapy with anti-fungal agents and corticosteroids, pachymeningitis showed regression. We hypothesised that intact immune status and less burden of Aspergillus infection in this patient may have resulted in a chronic progressive hypertrophic pachymeningitis.

Attention deficits in Japanese multiple sclerosis patients with minor brain lesion loads.

BACKGROUND: To investigate whether Japanese multiple sclerosis (MS) patients with minor brain lesion loads have attention deficits and brain atrophy, and to correlate their circumstance. METHOD: Twenty-one Japanese patients with relapsing-remitting MS were included in this study. Attention deficits were evaluated using Clinical Assessment for Attention (CAT) standardized according to age groups. Lesion load in the brain was assessed by tallying the total volume of plaques visible on brain magnetic resonance imaging (MRI). The width of the third ventricle and the bicaudate ratio were measured. RESULTS: The completion time for the visual cancellation tasks and/or the reaction times for the continuous performance test were prolonged in 14 patients (66.7%). The accuracy of responses was preserved throughout the CAT. Deviation from the normal value was not exaggerated based on the increasing difficulty of the task. The total volume of plaques on brain MRI was small. The width of the third ventricle was significantly increased in patients with MS when compared to controls, but was not correlated with the low performance on the CAT. CONCLUSIONS: Japanese MS patients with minor brain lesion loads frequently had attention deficits characterized by slowness of automatic information processing, but controlled processing that requires working memory demands was spared.

[Intramedullary spinal cord metastasis presenting with acute onset longitudinally extensive spinal cord lesion].

A 34-year-old previously healthy man presented with acute transverse lumbar myelopathy and MRI evidence of a longitudinally extensive spinal cord lesion (LESCL) from the upper thoracic cord extending down to the conus medullaris. Gadolinium-DTPA enhancement revealed a clearly demarcated enhanced nodule confined to the level of the 11th thoracic vertebral body (T11), which might have caused longitudinally extensive edema in the spinal cord. Histopathological appearance of adenocarcinoma of the nodule led to the ultimate diagnosis of lung cancer. Intramedullary spinal cord metastasis in a young patient without previously-diagnosed malignancy is a rare disorder, but should be listed up as a cause of LESCL.

[Progressive cerebral infraction initially presenting with pseudo-ulnar nerve palsy in a patient with severe internal carotid artery stenosis].

A 63-year-old man with hypercholesterolemia developed sensory and motor disturbances in the ulnar side of the right hand, and over three days the weakness evolved to entire right arm. Examination on the 6th day after onset showed mild lower facial palsy in addition to the upper limb weakness on the right. The weakness involved entire right arm sparing shoulder girdle muscles, which was worse in the 4th and 5th digits with claw hand deformity of the hand. Magnetic resonance imaging showed multiple small infracts in the centrum semiovale as well as in the medial side of the precentral knob on the left. Magnetic resonance angiography, ultrasonography, and 3D-CT angiography of the neck showed severe stenosis associated with unstable plaque of the left internal carotid artery. Hemodynamic mechanisms including microemboli and hypoperfusion associated with severe internal carotid artery stenosis are likely to cause stroke in evolution after initial presentation of pseudo-ulnar palsy in the present case.

Kana versus kanji in amyotrophic lateral sclerosis: a clinicoradiological study of writing errors.

The frequency of writing errors in samples from 14 patients with amyotrophic lateral sclerosis without manifest aphasia were compared with clinical background and indices from X-ray computed tomography, including the Evans' index (EI) and the cella media index (CMI). The inferior horn index (IHI) was measured as the maximal width of the short axis of the bilateral inferior horn of the lateral ventricles/the maximum transverse distance between the two internal laminae. Overt dementia and disinhibitive behavioral changes were significantly associated with frequency of total errors (p = 0.0280) and kanji errors (p = 0.0025). Significant associations were found for the EI with kana errors (p = 0.0481) and for the IHI with kanji errors (p = 0.0052). Preferential involvement of kana and kanji may reflect involvement of language-related areas in the frontotemporal lobes with frontal lobe or temporal lobe predominance.

Recurrent abducens nerve palsy associated with neurovascular compression.

We report a 50-year-old man who suffered from 5 transient diplopia episodes in 16 months. His diplopia lasted between 2 weeks and 3 months and examination revealed isolated left abducens palsy during the attacks of diplopia. Magnetic resonance (MR) angiography and MR imaging with constructive interference in the steady state sequence showed neurovascular compression of the left abducens nerve at the point of exit from the brain stem. Together with a lack of the preceding headache or febrile illness, we propose that neurovascular compression is a possible etiology of recurrent, isolated abducens nerve palsy.

[Herpes zoster oticus-associated jugular foramen syndrome].

We report a patient with herpes zoster oticus who presented with acute jugular foramen syndrome, and we present the review of 9 similar cases reported previously. Jugular foramen syndrome associated with varicella-zoster virus (VZV) infection is characterized by acute-onset dysphagia and dysphonia, usually accompanied or preceded by cranial, cervical, or pharyngeal pain. Herpetic eruptions on the skin or the mucosal surface may not occur, occur late after onset, or go undetected. Magnetic resonance imaging may reveal contrast enhancement around the jugular foramen, which implying inflammation of the glossopharyngeal or the vagal nerve ganglia, and its extension to the spinal root of the accessory nerve through the cerebrospinal fluid (CSF). The presence of VZV-DNA or VZV antibody in the CSF should be tested for early diagnosis and initiation of anti-viral treatment.

Ulnar entrapment neuropathy along the medial intermuscular septum in the midarm.

We report a patient with primary ulnar entrapment neuropathy in the midarm. Stimulation of multiple sites along the ulnar nerve showed a motor conduction block at a distance of 7.5-10 cm proximal to the medial epicondyle, where the nerve was compressed by the medial intermuscular septum. Anatomically, the possibility of ulnar nerve entrapment in this segment has long been suggested, and stimulation at least 10 cm above the medial epicondyle may reveal the entrapment. Muscle Nerve 39: 707-710, 2009.

Overnight switching from ergot-derived dopamine agonists to pramipexole in patients with Parkinson's disease: an open preliminary trial in Japan.

The aim of this study was to evaluate the safety and tolerability of overnight switching from ergot-derived dopamine agonists such as cabergoline to equivalent doses of pramipexole in patients with Parkinson's disease. The safety of overnight switching to pramipexole from cabergoline, which has a long plasma half-life and may cause dopaminergic excess after switchover, has not been established. Twenty-two consecutive patients with Parkinson's disease were included, 18 of them on cabergoline treatment. Patients were assessed with the Unified Parkinson's Disease Rating Scale (UPDRS) given just prior to switching as well as after 2, 4, 8, and 12 weeks of treatment. Eight patients (36.4%) experienced adverse events, of whom two were withdrawn from the study. Generally, however, significant improvement in the UPDRS was obtained after 2 weeks and improvement was maintained up to 12 weeks of treatment. Therefore, our study showed that overnight switching from ergot-derived dopamine agonists including cabergoline to dose-equivalent pramipexole was safe when associated with good patient compliance.

[Basilar artery occlusion with benign outcome--two case reports and a review on the role of collateral circulation and therapeutic time window].

We reported two cases of acute basilar artery occlusion (BAO) with favorable prognosis and discussed the role of collateral circulation. Patient 1 presented with minor brainstem dysfunction for 24 hours due to a short-segment embolic BAO at the mid-pontine level. Carotid angiogram demonstrated reversed basilar flow through the posterior communicating artery distal to the occlusion. Subsequently, the patient suddenly went into coma and developed tetraplegia due to spontaneous displacement and lodging of the embolus to the top of the basilar artery. Immediate recanalization was achieved by intra-arterial thrombolysis, and she recovered and was independent at 3 months after onset. Patient 2 developed progressive brainstem and cerebellar dysfunction due to thrombotic occlusion of the intracranial vertebral and the proximal basilar artery. Angiographic studies demonstrated that reversed basilar flow from the carotid system and meningeal anastomosis arising from the proximal vertebral artery filled the basilar artery distal to the occlusion. The patient recovered after conservative treatment leaving only residual signs of lateral medullary infarction. Recent case series show varied prognosis of BAO. Individual differences in the effectiveness of collateral circulation may be one of the reasons that accounts for this variability. The interval of reversible brainstem ischemia supported by the collaterals may widen the therapeutic time window up to recanalization following acute basilar artery occlusion.