Subcortical vascular dementia (SVD) exhibits relatively uniform clinical and pathological features among various subtypes of vascular dementia, and constitutes approximately half of vascular dementia in Japan. This subtype is further classified into Binswanger's type infarction and multiple lacunar infarctions. The former is characterized by diffuse white matter lesions, and the latter by lacunar infarctions. However, the both have common pathologic mechanism related to hypertensive small vessel changes. This paper overview the notion, clinical features, pathogenesis and treatment for SVD, and also refer to the recent topics related to Alzheimer's disease.
Our aim is to investigate the utility of transcranial Doppler (TCD) monitoring during intravenous administration of 0.6mg/kg recombinant tissue plasminogen activator (IV rt-PA) which is governmental approved in Japan. Acute ischemic stroke patients with M1 portion of the middle cerebral artery (M1) occlusion treated with IV rt-PA were prospectively enrolled. M1 occlusion was diagnosed before IV rt-PA using magnetic resonance angiography (MRA). Patients without sufficient temporal window of TCD were excluded. TCD monitoring was conducted for 1 hour (h) during IV rt-PA. Recanalization on TCD was defined using thrombolysis in brain ischemia (TIBI) flow grades. After all patients were classified into two groups according to the presence of TCD recanalization (TCD recanalization and TCD non-recanalization group), three-month patients outcome, recanalization rate on MRA 1 h of IV rt-PA, and symptomatic cerebral hemorrhage within 24 h were compared between two groups. We enrolled 16 patients. Eight patients (50%, 7 men [88%]; age, 70 years [interquartile range. 55-81]; NIHSS score, 18 [12-22]) were in the TCD recanalization group and 8 (50%, 6 men [75%]; age, 72 years [62-79]; NIHSS score 19 [15-23] were in the TCD non-recanalization group. Symptomatic cerebral hemorrhage was not seen in both groups at all. MRA 1 h of IV rt-PA revealed recanalization in all 8 (100%) patients with TCD recanalization group and 2 (25%) with TCD non-recanalization group (agreement, 88%; and kappa value, 0.75, P=0.002). At three months, 5 (63%) of 8 patients in the TCD recanalization group had favorable outcome, and 0 (0%) of 8 in the TCD non-recanalization group (P=0.026). TCD monitoring for 1 h during IV rt-PA can diagnose the recanalization based on MRA. TCD monitoring should predict good clinical outcome at three months.
The patient, a 63-year-old man, experienced the subacute onset of chorea, for which his family doctor prescribed oral haloperidol. However, the involuntary movements gradually worsened, and the patient was referred and admitted. High-signal lesions were seen in the caudate nucleus, putamen and globus pallidus bilaterally on MRI T2-weighted and FLAIR images. Chest CT, FDG-PET and tissue biopsies also revealed that the patient had lung adenocarcinoma with multiple lymph node metastases. The patient was diagnosed as having paraneoplastic chorea associated with primary lung adenocarcinoma. Antineuronal antibodies, such as anti-CRMP-5 and anti-Yo antibodies, were absent. The patient received steroid pulse therapy, oral prednisolone therapy, and concurrent radiochemotherapy. Chorea and high-signal lesions in the corpus striatum bilaterally on MRI improved quickly, and the mediastinal lymph node swelling also improved. The patient has been stable for 3 years since the onset of his symptoms. As the prognosis of paraneoplastic chorea is relatively favorable in some patients, it should be considered in the differential diagnosis of patients with chorea.
A 41-year-old man with multiple motor neuropathy developed weakness of the left hand at the age of 35 years. The weakness gradually progressed to his right hand. High-dose intravenous immunoglobulin (IVIg) therapy (0.4g/kg for 5 consecutive days) improved the muscle weakness in the hands but led to the development of generalized severe pompholyx that spread to the skin over the entire body. Because muscle weakness of the hands worsened several months after IVIg therapy, we attempted another course of IVIg therapy. However, anti-allergic agents and oral corticosteroids did not suppress the pompholyx induced by the high-dose IVIg. Hence, the treatment was switched to low-dose immunoglobulin therapy (0.4g/kg for one day) once every month. After more than 8 months of low-dose therapy, only mild form of pompholyx remained and the muscle strength was maintained without further deterioration.
A 16-year-old woman was admitted to our hospital because of the motorbike accident. On admission, her consciousness was alert, and she had lower lip laceration and left femur open wound. Brain CT showed no abnormality, but chest CT revealed slight left lungs sprain and her left femur was fractured in the radiograph. Debridement and the art of the steel wire pulling of the left femur fracture part were enforced under general anesthesia about two hours coming to a hospital later. Her awaking from anesthetizing after the operation was delayed, and the consciousness level gradually decreased. Head MRI diffusion weighted image (DWI) after about 36 hours coming to a hospital showed diffuse high signal region centering on the cerebral white matter. We diagnosed her as having cerebral fat embolism based on clinical course and MRI findings. DWI abnormal signals disappeared in three weeks and the symptoms had improved gradually. It is thought that head MRI-DWI is useful to diagnose and evaluate the pathophysiology of cerebral fat embolism.
A 73-year-old man, a right-handed, has been pointed out his atrial fibrillation and seen a doctor regularly for varicose veins of left leg and hypertension. He had complaint of a sudden paralysis of his left thumb and index finger while drinking beer. The next day, there was no improvement and he was admitted to our hospital. Neurological examination revealed mild weakness of the most muscles of both thumb and index finger which were innervated by radial nerve, ulner nerve, and median nerve. But he had no any other neurological deficits including sensory system. A brain MRI revealed the acute-stage cerebral infarction in the right precentral knob. With other examinations, we diagnosed the cerebral infarction as cardiogenic embolism. Pure motor isolated finger palsy (PMIFP) in association with cerebrocortical small lesion is rare. It is probable that some cases with diagnosed of peripheral neuropathy was actually PMIFP from central nervous system disturbance.
We report the beneficial and adverse effects of modafinil for daytime sleepiness in a 62-year-old female patient with myotonic dystrophy. Although it was effective for excessive daytime sleepiness, orolingual dyskinesia appeared the day following administration of modafinil (100mg/day), and dyskinesia disturbed her daily life including dental treatment. When modafinil was stopped, dyskinesia was improved. However, excessive daytime sleepiness deteriorated gradually; re-treatment with smaller dosage (50mg every other day) resulted in partial improvement but aggravation of both dyskinesia and diabetes mellitus. Modafinil should be administered carefully when the patient is older or has complications such as glucose intolerance.
We reported a 61-year-old man who had developed acute cerebellar ataxia in the trunk and the lower limbs. His chemical blood analysis showed very mild hypothyroidism and the presence of serum anti-thyroid peroxidase (TPO) antibody and anti-NH2 terminal of alpha-enolase (NAE) antibody. While cerebellar atrophy was not evident on magnetic resonance imaging (MRI) of the brain, 99mTc-ECD SPECT using the easy Z-score imaging system (e-ZIS) showed decreased regional cerebral blood flow (rCBF) in the vermis of cerebellum. His cerebellar ataxia improved spontaneously within three weeks. The present case is very rare and suggests that anti-NAE autoantibody may be associated with actue cerebellar ataxia.
A previously healthy, 10-year-old girl developed left optic neuritis that treated with oral prednisolon (PSL). During the following 8 months, the patient exhibited right optic neuritis 3 days after discontinuation of PSL therapy and three episodes of epileptic seizures 3 weeks after PSL withdrawal. Cerebrospinal fluid (CSF) examination revealed pleocytosis (mononuclear cells), increased IgG index, and positive oligoclonal IgG expression. Brain MRI showed multiple cortical, subcortical, and leptomeningeal enhanced lesions. However, spinal cord MRI revealed no lesions. Neither autoantibodies to nuclear, thyroid, α-enolase, glutamic acid decarboxylase, nor aquaporin 4 was detected. However, anti-NMDA receptor antibodies (NMDAR-Ab) were present in her CSF. This patient is the second reported case of NMDAR-related encephalitis with recurrent optic neuritis. The possibility of seronegative neuromyelitis optica (NMO) could not be ruled out for the symptom of recurrent optic neuritis. However, the presence of NMDAR-Ab in the CSF together with increased IgG index and oligoclonal IgG bands, which are usually negative in NMO suggested that this patient is NMDAR-related encephalitis combined with rare symptom of optic neuritis for this type of encephalitis, though we need to wait larger number of patients' accumulation to conclude that the optic neuritis could be one of the features of NMDAR-related encephalitis.