Efficacy of Early Superficial Temporal Artery-Middle Cerebral Artery Double Anastomoses for Atherosclerotic Occlusion in Patients with Progressing Stroke.

BACKGROUND: We investigated the efficacy of early superficial temporal artery-middle cerebral artery (STA-MCA) double anastomoses for patients with progressing stroke due to atherosclerotic occlusion. MATERIALS AND METHODS: Nine consecutive patients who underwent early STA-MCA double anastomoses were enrolled. All patients presented with progressing stroke despite maximal medical treatment. Cerebral blood flow in 7 patients was analyzed by single-photon emission tomography. Clinical outcomes were investigated postoperatively, and we evaluated the utility of early STA-MCA double anastomoses. RESULTS: Nine patients in the present study included those with middle cerebral artery occlusion (n = 6) and internal carotid artery occlusion (n = 3). The mean age was 58.4 years. Subjects comprised 1 female (11.1%) and 8 males (88.9%). The cause was low perfusion ischemia due to atherosclerotic occlusion with a small infarct. The mean regional cerebral blood flow (rCBF) ratio in the middle cerebral artery territory compared to the normal side was 69.6 ± 5.3%. The duration from onset to surgery was 1-8 days (median, 3.11 days). All patients underwent early STA-MCA double anastomoses, and no reperfusion-induced hemorrhage occurred. All of them slowly achieved obvious remission compared to symptoms on admission and achieved a good functional outcome. CONCLUSIONS: Early STA-MCA double anastomoses were safe and effective, and early revascularization resulted in rapid neurological improvement. We recommend this procedure for patients with progressive ischemia due to main trunk artery occlusion, when the rCBF flow ratio with the normal side was 70 ± 10%, even at the subacute stage.

Apathy and depression in Parkinson disease.

The purpose of this study was to investigate the prevalence and clinical correlates of apathy and depression in Parkinson disease (PD), and to clarify whether apathy can be dissociated from depression. One hundred fifty patients with PD completed the Beck Depression Inventory Second Edition (BDI-II), Starkstein's Apathy Scale (AS), and a quality of life (QOL) battery. Hoehn and Yahr (HY) staging, the Unified Parkinson's Disease Rating Scale (UPDRS), and the Mini-Mental State Examination (MMSE) were performed on the same day. Apathy (AS score > or = 16) was diagnosed in 60% of patients and depression (BDI-II score > or = 14) in 56%. Apathy coexisted with depression in 43% of patients, compared with depression without apathy in 13% and apathy without depression in 17%. Apathy scale score was significantly correlated with UPDRS scores, HY stage, and age, whereas BDI-II score was correlated only with UPDRS scores. Both AS and BDI-II scores were negatively correlated with QOL. However, multiple regression analysis revealed that depression was strongly and negatively associated with emotional well-being and communication, whereas apathy was mainly associated with cognition and stigma. These findings suggest that apathy and depression may be separable in PD, although both are common in patients with PD and are associated with QOL.

[A case of progressive supranuclear palsy with late-onset supplementary motor area seizure].

We report a 75-year-old, right-handed man, presenting with supplementary motor area (SMA) seizure. The patient had suffered from frequent attacks of transient inability to speak and move without loss of awareness. On admission, he presented with vertical gaze paresis, axial rigidity, paratonia of extremities and gait disturbance. The attacks were preceded by discomfort on the head, followed by inability to move the whole body and arrest of vocalization with tonic posture and exaggerated breathing. Consciousness and cognitive function were preserved throughout the attacks. Electroencephalography recorded intermittently slow theta waves in the bifrontal regions. Brain MRI showed atrophy of the midbrain tegmentum with lacunar state suggesting progressive supranuclear palsy. SPECT with 123I-iomazenil revealed decreased uptake in the medial frontal areas including SMA, bilaterally. The seizures resolved completely following treatment with carbamazepine. Based on clinical features and neuroimagings, we speculated that the negative motor area within SMA was responsible for his seizure. Physicians should keep in mind that SMA seizure comprising negative motor phenomenon can occur in the elderly.

[Case of multiple sclerosis with WEBINO syndrome].

We report a 50-year-old man presenting with wall-eyed bilateral internuclear opthalmoplegia (WEBINO) syndrome. He had suffered from progressive double vision and tetraparesis, and been diagnosed as secondary progressive multiple sclerosis (MS). On admission, he presented with bilateral facial nerve palsy, pseudobulber palsy, and spastic tetraparesis, predominantly on the right side. Bilateral adduction deficits were noted on horizontal gaze, together with nystagmus of abducting eyes. On primary eye position, the right eye was fixed in the midposition, while the left eye was exotropic. The right eye was deviated outward on fixation with the left eye. Vertical gaze and convergence were preserved. These ocular findings were compatible with WEBINO and considered to result from impairment of bilateral medial longitudinal fasciculus and imbalance of paramedian pontine reticular formation on both sides. T2-weighted images of MRI revealed a high signal lesion in the paramedian pontine tegmentum without enhancement. He underwent steroid pulse therapy, followed by mild improvement in adduction of both eyes. Although WEBINO tends to be observed in the acute stage of stroke, this patient suggests that demyelinative lesions of MS can cause persistent WEBINO, involving the paramedian pontine tegmentum.

[A case of Creutzfeldt-Jakob disease with stroke-like episode as an initial symptom].

A 79-year-old woman was admitted to our hospital, due to acute onset of left hemiparesis and disturbance of consciousness. Although her symptoms improved temporarily, she developed gait disturbance and cognitive deterioration 2 months after the onset. After that, she presented with myoclonus and startle response, followed by akinetic mutism within 8 months after the onset. Serial EEGs revealed no periodic synchronous discharge. Serial diffusion-weighted MRIs showed that high intensity lesions, which initially limited to the right cerebral cortex, gradually spread to the bilateral cerebral cortices and basal ganglia, with relative sparing of central gyri, medial occipital cortices, and hippocampus. Prion protein gene analysis revealed a point mutation (Val-->Ile) at codon 180. The result of this patient suggests that this type of CJD might be associated with an atypical clinical course such as stroke-like episode and selective involvement of cortical and subcortical lesions.

[Pulseless arrest in an elderly patient treated with intravenous tissue plasminogen activator for cardioembolic ischemic stroke].

A 77-year-old woman, who had a history of rheumatic mitral stenosis with atrial fibrillation (AF), was referred and admitted to our hospital because of a transient ischemic attack at 4: 55 p.m.. She had taken warfarin for over 10 years, but her condition was not well controlled on admission. At 8: 30 p.m., she had acute ischemic stroke with right facial palsy, right hemiparesis and slurred speech. At 10: 35 p.m., she was treated with intravenous tissue plasminogen activator (t-PA) and her neurological deficits almost fully recovered by 0: 05 a.m. (90 min after t-PA started). At 0: 08 a.m., she collapsed due to sudden pulseless arrest. Using advanced life support, she soon recovered with no complications. After mitral valve replacement and left atrial appendectomy, she was discharged with a modified Rankin scale 0 at day 40. To the best of our knowledge, this is the first case report showing pulseless arrest immediately after treatment with t-PA in an elderly patient with ischemic stroke. Left insular injury seemed to be a crucial mechanism of pulseless arrest in this case.

Primitive reflexes distinguish vascular parkinsonism from Parkinson's disease.

OBJECTIVES: Although vascular parkinsonism (VP) occurs frequently in the elderly, its clinical features have not been investigated in detail, particularly in comparison with Parkinson's disease (PD). The goal of this study is to clarify the diagnostic value of pathological reflexes in differentiating between VP and PD. PATIENTS AND METHODS: In 132 patients with PD and 55 with VP, pathological reflexes, including snout reflex (SR), palmomental reflex (PMR), corneomandibular reflex (CMR), jaw reflex (JR), Hoffmann reflex (HR), and extensor plantar response (EPR), were evaluated. RESULTS: The percentage of each pathological reflex elicited in two groups (VP:PD) was as follows: SR (78:30), PMR (53:26), CMR (9:6), JR (33:12), HR (29:11), and EPR (25:8). The prevalence of pathological reflexes, except for CMR, was significantly higher in the VP patients than in the PD patients. In particular, SR and PMR were more frequent than upper motor neuron signs in the VP patients. The sensitivity and specificity of either SR or PMR for VP were 84% and 82%. CONCLUSION: Snout and palmomental reflexes are useful tools in the differentiation between VP and PD.

[Case of Hashimoto's encephalopathy with diffuse white matter lesions on diffusion-weighted MRI].

A 65-year-old woman was admitted to our hospital because of subacute deterioration of cognitive function. On admission, she presented with marked disorientation of time and place and inability to carry out commands. Mini-Mental State Examination score was 5/30. Although routine laboratory examinations including thyroid function, vitamin B1 and B12, serum syphilitic reaction, sIL-2 receptor level, titers of herpes simplex and zoster viruses, and HIV antibody were normal, titers of anti-thyroglobulin (TG) antibodies and thyroid peroxidase (TPO) antibodies were elevated. Cerebrospinal fluid showed normal findings. Brain MRI revealed diffuse high intensity in the white matter on diffusion- and T2-weighted images, mimicking leukoencephalopathy. We made a diagnosis of Hashimoto's encephalopathy, based on clinical features and high titers of anti-thyroid antibodies. Following administration of steroid hormone, her cognitive impairment gradually improved, associated with decrease of the white matter abnormality on MRI. Hashimoto's encephalopathy should be kept in mind in the differential diagnosis of subacute leukoencephalopathy with cognitive decline.

[A case of hereditary sensory and autonomic neuropathy type 1E with frontal lobe dysfunction as an initial symptom].

A 49-year-old man had developed gradually personality change, gait disturbance, and hearing loss for five years. On admission, he presented with frontal release signs, stuttering, vertical gaze palsy, sensorineural deafness, muscle rigidity, ataxia, and sensory disturbance with areflexia in the lower extremities. Brain MRI demonstrated atrophy in the cerebellum and midbrain tegmentum as well as cerebral atrophy, predominantly in the frontal lobe. He was tentatively diagnosed as progressive supranuclear palsy on the basis of clinical features and imagings. On nerve conduction study, no sensory nerve action potentials were elicited in the upper and lower extremities. Details of family history revealed a hereditary sensory neuropathy with autosomal dominant inheritance in his relatives. Because genetic analysis showed a rare missense mutation (c.1483T>C, p.Y495H) in DNA methyltransferase 1 gene, we diagnosed him as having hereditary sensory and autonomic neuropathy type 1E (HSAN1E). In addition, p.M232R mutation in prion protein gene was detected. It should be kept in mind that there are some patients with HSAN1E presenting with frontal lobe dysfunction as an initial symptom and with clinical features mimicking progressive supranuclear palsy.

Usefulness of cervical computed tomography and magnetic resonance imaging for rapid diagnosis of crowned dens syndrome: A case report and review of the literature.

INTRODUCTION: Crowned dens syndrome is a rare disease entity which radiologically shows calcification of the cruciform ligament around the odontoid process. We report a patient with crowned dens syndrome who improved dramatically in 5days following treatment with oral nonsteroidal anti-inflammatory medication. PRESENTATION OF CASE: A 61-year-old man was admitted to our hospital with a severe occipital headache and sudden onset of neck stiffness. Neurological examination on admission revealed a high fever and cervical rigidity. Laboratory examination revealed a markedly elevated white blood cell count and C-reactive protein level, but cerebrospinal fluid studies revealed only a slight abnormality. A cervical computed tomography scan and its three-dimensional reconstruction detected a remarkable crown-like calcification surrounding the odontoid process. Cervical magnetic resonance imaging did not demonstrate strong direct compression of the cervical cord; however, the soft tissue surrounding the odontoid process was hyperintense on T2-weighted imaging with fat suppression. Based on the radiological findings, the patient was diagnosed with crowned dens syndrome and was immediately treated with non-steroidal anti-inflammatory drugs. The patient's condition drastically improved within 5days. DISCUSSION: It was very interesting that the soft tissue surrounding the odontoid process was hyperintense on magnetic resonance T2-weighted imaging with fat suppression, and the signal change disappeared 2 weeks after the administration of oral non-steroidal anti-inflammatory drugs. We think that magnetic resonance imaging is useful for proving inflammation in patients with crowned dens syndrome. CONCLUSION: This is the first report making reference to the magnetic resonance imaging findings of crowned dens syndrome.

[A case of high-aged idiopathic spinal cord herniation from dural defect].

Idiopathic spinal cord herniation was assumed to be a rare disease. However, the incidence of discovering this condition appears to have been increasing recently with advances in neuroradiological diagnosis using magnetic resonance imaging (MRI) and computed tomographic myelogram (CTM). We present herein an operated case of high-aged idiopathic spinal cord herniation. A 71-year-old female presented with spastic paresis of the right lower limb and sensory disturbance of pain and temperature below the level of the left Th4 dermatome, consistent with Brown-Séquard syndrome. MRI and CTM revealed right ventral displacement of the spinal cord and dilatation of the dorsal subarachnoid space at Th2/3. Laminectomy of Th1-3 was performed, herniated spinal cord was untethered and repositioned, and the dural defect was sealed with GORE-TEX dura substitute. Postoperative MRI revealed normal location of the spinal cord and neurological state improved slightly. The patient was discharged 1 month after the operation. Among many cases of spinal cord herniations, this is considered to be a rare case of idiopathic spinal cord herniation in an elderly patient.

[A clinicoanatomical study of thalamic cheiro-oral syndrome].

We report 6 patients with Cheiro-oral syndrome (COS), with special reference to clinical features and responsible lesions. The time intervals from the onset of symptoms to arrival in our department were less than 24 hours in 3 patients, 2 days in 2, and 5 days in 1. All patients had subjective sensory disturbance involving the unilateral hand and ipsilateral perioral regions, and 4 patients presented with objective sensory disturbance. The body parts of tingling sensation tended to be larger than those of superficial sensory disturbance. Three patients developed motor disturbance including hemiparesis with or without ataxia, clumsiness of fine finger movements, and dysarthria. Magnetic resonance imaging revealed fresh infarctions around the thalamus, including lacunar infarctions in 5 patients and branch atheromatous disease in 1 patient. The lesion sites responsible for COS were ventral posterolateral (VPL) and ventral posteromedial (VPM) nuclei in the thalamus in 4 patients, thalamic pulvinar nucleus and medial geniculate body in 1, thalamic ventroposterior region-internal capsule-corona radiata in 1. Three patients had asymptomatic brain infarctions. Risk factors were hyperlipidemia, hypertension, diabetes mellitus, smoking, arteriosclerosis of the carotid artery, and polycythemia. In the convalescent stage, 5 patients suffered from residual sensory-motor disturbance, whereas 1 patient recovered from COS. COS has been attributed mainly to small infarctions in the thalamic ventroposterior nuclei. However, it is suggested that damage to ascending sensory fibers projecting to the thalamic VPL and VPM nuclei can cause COS. Because initial symptoms of COS are apt to be overlooked, early diagnosis and treatment are necessary to avoid residual sensory-motor disturbance.

Importance of perioperative management for emergency carotid artery stenting within 24h after intravenous thrombolysis for acute ischemic stroke: Case report.

INTRODUCTION: We report a patient treated successfully via endovascular surgery within 24h after intravenous thrombolysis using recombinant tissue plasminogen activator for acute cervical internal carotid artery occlusion. PRESENTATION OF CASE: A 68-year-old man was admitted to our hospital. Neurological examination revealed severe left-sided motor weakness. Magnetic resonance imaging showed no cerebral infarction, but magnetic resonance angiography revealed complete occlusion of the right internal carotid artery. Systemic intravenous injection of recombinant tissue plasminogen activator was performed within 4h after the onset. But, magnetic resonance angiography still revealed complete occlusion. Revascularization of the right cervical internal carotid artery was performed via endovascular surgery. The occluded artery was successfully recanalized using the Penumbra System(®) and stent placement at the origin of the internal carotid artery. Immediately after surgery, dual antiplatelet therapy (aspirin and clopidogrel) was initiated, and then cilostazol was added on the following day. Carotid ultrasonography and three-dimensional computed tomographic angiography at 14days revealed no further obstruction to flow. DISCUSSION: When trying to perform emergency carotid artery stenting within 24h after intravenous recombinant tissue plasminogen activator administration, several issues require attention, such as the decisions regarding the type of stent and embolic protection device, the selection of antiplatelet therapy and the methods of preventing hyperperfusion syndrome. CONCLUSION: Emergency carotid artery stenting for the acute internal carotid artery occlusion may be considered a safe procedure in preventing early stroke recurrence in selected patients.

[A case of multiple sclerosis with bilateral useless hand syndrome as a main clinical feature].

A 41-year-old, right-handed man was admitted to our hospital on September 12, 2002, due to progressive clumsiness in both hands. The patient had been diagnosed as having multiple sclerosis three years prior to admission. He noticed difficulty in manipulating objects three months before admission. Cervical T2-weighted MRI showed a high signal intensity at the level of C3-4 which was enhanced on T1-weighted image with gadolinium. On admission, neurological examinations revealed impairment of dexterity, deep sensory disturbance, and astereognosis in both hands. The clumsiness of complex finger movements was predominant on the left side, and was exaggerated with the eyes closed in association with pseudoathetosis. After steroid therapy, his clumsy hands improved gradually. This type of clumsiness in multiple sclerosis had been described as useless hand syndrome by Oppenheim. In accordance with our case, useless hand syndrome has been reported to arise from high cervical (C2-4) lesions, mainly involving the posterior cord ipsilateral to the clumsy hand. Although the majority of reported cases with useless hand syndrome had other neurological complications, such as hemiparesis, tetraparesis, and truncal ataxia, our patient exhibited a pure form of useless hand syndrome. In addition, useless hand syndrome is usually unilateral, and bilateral useless hand syndrome is very rare. Clumsiness of fine finger movements with astereognosis in our patient is similar to numb clumsy hands or limb-kinetic apraxia due to cervical spondylosis or postcentral gyrus lesion, respectively. This indicates an important role of the high cervical posterior cord in conveying a kinesthetic sense necessary to guide fine finger movements. It should be kept in mind that high cervical lesions in multiple sclerosis causes clumsy hands mimicking limb-kinetic apraxia.

Useless hand syndrome with astereognosis in multiple sclerosis.

We report on a patient with multiple sclerosis (MS) who developed bilateral useless hand syndrome (UHS) and astereognosis. Clinical features of UHS in our patient are similar to limb-kinetic apraxia with astereognosis. Cervical T1-weighted magnetic resonance imaging revealed a gadolinium-enhanced lesion at the level of C3-C4, mainly involving the posterior cord. This patient suggests that posterior cord lesions at high cervical levels cause UHS and astereognosis in MS, involving the sensorimotor integration needed to execute complex finger movements.

Fulminant Guillain-Barré syndrome after Campylobacter jejuni enteritis and monospecific anti-GT1a IgG antibody.

A 21-year-old man developed rapid progression of tetraplegia, bulbar palsy, and respiratory paralysis after Campylobacterjejuni enteritis. Based on the diagnosis of Guillain-Barré syndrome, he received plasmapheresis and intravenous immunoglobulin. Serum anti-GT1a IgG antibody which lacked cross-reactivity with GQ1b was detected. Four months after the onset, the patient still had severe muscle weakness of the lower limbs. This case suggests that anti-GT1a IgG antibody can be associated with severe paralysis in Guillain-Barré syndrome after C. jejuni enteritis.

Anterior cerebral artery territory infarctions presenting with ascending tetraparesis.

We describe a patient with ascending tetraparesis following stroke. The patient presented initially with spastic paraparesis which acutely evolved to tetraparesis with abulia. Magnetic resonance imaging revealed acute infarctions in the bilateral medial frontal regions but not in the brainstem or spinal cord. Multiple infarctions in the anterior cerebral artery territory appeared to originate from artery to artery embolism. The present case provides distinct clinical features of anterior cerebral artery syndrome which mimic myelopathy or brainstem lesions.

[A case of acute measles encephalitis with periodic synchronous discharge on electroencephalography].

A 18-year-old man was diagnosed as having measles on the basis of cutaneous and mucosal eruption and high grade fever on May 10, 2001. Six days after the skin eruption, the patient developed general convulsion (day1). He was admitted to our hospital because of status epilepticus. We made a diagnosis of acute measles encephalitis, based on the clinical features and pleocytosis with an increase in protein in the cerebrospinal fluid. Under artificial ventilation and sedation, he received intravenous immunoglobulin and dexamethasone. Electroencephalography (EEG) on day 4 revealed periodic synchronous discharge (PSD). Significant elevation of antibody titer for measles virus was found in the serum, but not in the cerebrospinal fluid. Polymerase chain reaction method did not show viral genes of measles virus, herpes simplex virus and herpes zoster virus. Serial EEG studies demonstrated a decrease in PSD, followed by irregular spike-wave complexes within 20 days. He recovered completely one month after the onset. It should be kept in mind that PSD can emerge on EEG in the early stage of acute measles encephalitis.

[A case of motor neuron disease with dementia and apraxia of the upper limbs].

We report a 61-year-old, right-handed woman with motor neuron disease, dementia, and apraxia of the upper limbs. The patient developed clumsiness of the right hand and dysarthria two years and a half prior to admission. Neurological examination showed limb-kinetic apraxia and ideomotor apraxia, predominantly on the right side, in addition to dementia and anarthria. There was mild muscle wasting in the neck and hands. A muscle biopsy from the biceps muscle of arm as well as needle EMG revealed neurogenic changes compatible with motor neuron disease. Brain MRI indicated pyramidal tract degeneration. Three-dimensional brain perfusion imaging generated from SPECT demonstrated an asymmetric decrease in cerebral blood flow in the fronto-temporo-parietal regions, predominantly on the left side. This case suggests that asymmetric limb apraxia can be associated with motor neuron disease.

[A case of pure autonomic failure (PAF) with sleep apnea syndrome (SAS) and successful treatment of dysautonomia with nasal continuous positive airway pressure (CPAP)].

A 72-year-old man was admitted to our hospital due to dysuria and frequent syncope. The patient had been well until the age of 70 years, when he began with these symptoms and neurogenic bladder was diagnosed in the other hospital. On admission, neurological examinations revealed no abnormal findings except blepharoptosis, anisocoria and orthostatic hypotension. Frequent apnea was evident during sleep. Autonomic function tests showed mainly sympathetic postganglionic dysfunction. Brain magnetic resonance imaging showed lacunar infarctions without cerebello-pontine atrophy or abnormal signals of the basal ganglia. We diagnosed pure autonomic failure (PAF) with sleep apnea syndrome (SAS). After starting nasal continuous positive airway pressure (CPAP) for SAS, his sneezing and sleep apnea drastically improved. Interestingly, CPAP also decreased the severity of orthostatic hypotension and syncope. Ambulatory blood pressure monitoring (ABPM) showed remarkable improvement in diurnal fluctuation of blood pressure after CPAP therapy. Although SAS is frequently associated with Shy-Drager syndrome but not with PAF, patients with PAF had been reported to have degenerative changes in the central nervous system overlapping with Shy-Drager syndrome or Lewy body disease. This case raised the possibility that nasal CPAP may be useful for orthostatic hypotension as well as SAS in neurodegenerative diseases.