The cerebral cortex controls cognitive and voluntary process of movements. The brainstem and spinal cord are involved in the execution of innately acquired motor patterns such as postural reflexes, muscle tone regulation and locomotion. Cortico-reticular projections arising from the motor cortical areas contribute to the postural control that precedes the voluntary movement process. The basal ganglia cooperatively regulates the activities of the cerebral cortex and the brainstem-spinal cord by its strong inhibitory and dis-inhibitory effects upon these target structures so that goal-directed movements could be appropriately performed. We propose that basal ganglia dis-function, including the abnormality in the dopaminergic projection system, may disturb the cooperative regulation, resulting in motor deficiencies expressed in basal diseases.
The aim of this study is to investigate the frequency, causes, and clinical characteristics of individuals with presenile dementia with an age of onset less than 65 years. A staged questionnaire survey was performed among all hospitals and clinics, all faculties of care and welfare services, and all local governmental offices in Aichi prefecture. The response rate of the primary survey was 62.3%, and that of the secondary survey was 90.1%. The number of people with presenile dementia after adjusting for duplicated subjects was 1,092 (569 men, 520 women and 3 of unknown gender). The average age was 60.7±7.1 (mean±SD) years, and age of onset was 55.1±7.8 years. Vascular dementia (VD) was the most frequent cause in men (42.2%), followed by Alzheimer's disease (AD: 24.8%), frontotemporal dementia (FTD: 4.6%) and Parkinson disease (PD: 3.8%). In women, AD was the most common (45.8%), followed by VD (25.4%), FTD (7.4%) and PD (3.4%). Overall, AD and VD were the most common causes of presenile dementia, followed by FTD and PD. The highest prevalence of presenile dementia was seen in the age range of 60-64 years old. This was true for both men and women.
Purpose: To evaluate the development or worsening of myoclonus in patients receiving gabapentin (GBP). Methods: Clinical charts of 162 patients treated with GBP were reviewed concerning development or worsening of myoclonus. Results: We found 3 cases (1.9%) of myoclonus. Two patients had preexisting myoclonus and generalized tonic-clonic seizures, while the other one had generalized tonic-clonic seizures only. All patients experienced development or worsening of myoclonus within 2 weeks after starting GBP. Dose at the onset of development or worsening of myoclonus varied from 600mg to 1,800mg. Two patients developed multifocal myoclonus. Discontinuation of GBP or clonazepam add-on resulted in cessation of myoclonus with no serious sequela. Conclusion: GBP may increase the risk of development of de novo myoclonus or worsening of myoclonus in patients with preexistent myoclonus. According to the result of this study and the treatment guidelines, GBP should be avoided when a patient has preexistent myoclonus.
A 48-years old man presented with slowly progressive bradykinesia, personality change and rapidly progressive left hemiparesis. On admission, he presented dementia, poor judgment, left hemiparesis. MRI revealed a widespread high intensity area in right hemisphere and MRA was almost normal. Serological tests of serum and CSF demonstrated high titers of antibodies to Treponema pallidum. He was treated for syphilis with daily penicillin injections without improvement. He died of sepsis eight months after admission. At autopsy, the brain weighed 1,100g and the right cerebral hemisphere was atrophic, especially in frontal base, temporal, parietal, angular, and posterior regions covered by thickened, fibrotic leptomeninges. Microscopically, chronic meningoencephalitis was observed. Severe neuronal loss with gliosis was seen in the right cerebral cortices. Scattered rod-shaped microglia and inflammatory cell infiltration were visible in the cerebral parenchyma. The dorsal column of the spinal cord was not involved and meningovascular syphilis was unclear. The distribution of the encephalitic lesions was well correlated with the clinical and neuroradiological findings. This was a rare autopsy case presenting Lissauer's general paresis, clinically manifesting as rapidly progressive stroke-like episode.
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.
We report a 58-year-old woman with adult onset Alexander disease. At the age of 54 she noticed numbness in bilateral legs and at 57 she developed left sided spastic gait. Her walking difficulty was gradually worsened and followed by the development of weakness in left arm, dysarthria and dysphagia. Her mother and elder brother also had similar clinical presentations which suggested an autosomal dominant neurological disorder. With MRI findings showing localized atrophy of medulla oblongata and upper cervical cord with hyperintensities on T2-weighted image, diagnosis of adult onset Alexander disease was made. We performed genetic analysis and found novel variant (S398F) in the glial fibrillary acidic protein gene. In case of slowly progressive myelopathy with bulbar palsy of unknown origin, especially those with atrophy limited to medulla oblongata and upper cervical cord, adult onset Alexander disease should be taken into consideration.
A 55-year-old man complained of intermittent piercing pain on the right side of his throat. In a visit to our hospital, he was diagnosed with glossopharyngeal neuralgia and was treated with carbamazepine. But he obtained no pain relief and had drug-induced hypersensitivity syndrome due to carbamazepine on admission. We discontinued the carbamazepine, so the severity of the throat pain increased. We then administered codeine, which alleviated the pain; an increase in the dosage led to complete pain remission. After his general condition improved, he underwent an operation for microvascular decompression. In general, opioids are not efficacious against neuropathic pain, including glossopharyngeal neuralgia. However, recent studies do show their effectiveness against such pain via various mechanisms. They may control neuropathic pain through their effects on cortical brain regions and the thalamus, they may affect the descending antinociceptive pathway via actions on the periaqueductal gray, and they may modulate pain transmission in the spinal dorsal horn. We believe this to be the first case report to mention the effectiveness of opioids for glossopharyngeal neuralgia. If the control of pain is difficult in cases of glossopharyngeal neuralgia, opioids may be a useful therapeutic option.
A 75-year old man with multiple system atrophy received percutaneous endoscopic gastrostomy (PEG) because of dysphagia. But recurrent aspiration pneumonia occurred after PEG nutrition, which was due to gastroesophageal reflux. As he had floppy epiglottis, orally inserted endoscopic procedure caused upper airway obstruction, which required transient non-invasive positive-pressure ventilator (NIPPV) treatment. He underwent transgastrostomal jejunal tube (PEG-J) replacement under the nasal endoscopic guidance successfully, but tube was patent only for 5-months. Thereafter, endoscopic jejunostomy (PEJ) via gastric stoma was performed on NIPPV safely, and the patient is now stable. For the management of nutrition in the advanced stage of neurodegenerative disease patients, PEJ is one of useful choice.