A lesion responsible for central paroxysmal positional vertigo (PPV) is often found in the dorsolateral wall of the fourth ventricle. A relatively large tumor or hemorrhage in the dorsolateral wall of the fourth ventricle usually causes central PPV, but small brain infarction has hardly been reported to cause central PPV. We report three cases of a small brain infarction in the lateral wall of the fourth ventricle presenting with central PPV. All of the cases showed similar clinical features in which a given recumbent position and a sitting position caused a dizziness associated with nausea and vomiting over an acute period. The symptom lasted one to two months after the onset. In two of our cases, no neurological abnormal signs, except nystagmus, were observed, and initial differentiation of central PPV from peripheral PPV was difficult. Interruption of the vestibular nuclei-archicerebellar loop seems to be responsible for the central PPV. Recognition of the clinical features of central PPV and diffusion-weighted MRI images are important for a precise local diagnosis in small brain infarction showing PPV.
Influenza-associated encephalopathy (IAE) usually occurs in children aged <5 years. Adult cases of IAE are very rare and, thus far, no definite adult autopsy case has been reported. Here, we present the first definite adult autopsy case of IAE. A 76-year-old man presented with sudden coma a day after the onset of fever caused by infection with influenza type A virus. Soon after admission, his condition was complicated by DIC, shock, and multiple organ failure, and he was diagnosed with IAE. Oseltamivir administration and steroid pulse therapy were performed but these proved to be ineffective. The patient died about 24 hours after the onset of encephalopathy. The autopsy revealed massive brain edema and diffuse increase of amoeboid glias without inflammatory cell infiltration. Influenza type A/Hong Kong virus (H3) was isolated from his lungs. Serum IL-6 level was extremely high (35,800pg/ml; normal, 0.221-4.62pg/ml). The clinical course, and the laboratory and pathological findings of this adult case resembled those of a typical childhood-onset IAE, suggesting the same pathogenesis. During the influenza season, IAE should be taken into account for differential diagnosis in adult patients with altered mental status and fever.
We investigated retrospectively 35 patients with amyotrophic lateral sclerosis who underwent percutaneous endoscopic gastrostomy (PEG), focusing on their respiratory function at the time of PEG. There were statistically significant correlations between the forced vital capacity and arterial carbon dioxide pressure, and the duration from the PEG to death or initiation of ventilatory support. In comparison between the 20 patients who died or was placed on ventilator within 6 months after PEG (group A) and the 15 patients who survived more than 6 months without ventilatory support after PEG (group B), the arterial carbon dioxide pressure showed a significantly high level in the group A (mean 47.5±S.D. 5.4mmHg) than in the group B (42.2±5.2mmHg) (P < 0.05). PEG should be performed in ALS patients while respiratory function is well preserved, especially before arterial carbon dioxide pressure is increased.
Juvenile myoclonic epilepsy (JME) is one of the most representative idiopathic generalized epilepsies occurring in adolescence. Although epileptic seizures in JME are easily controlled by appropriate antiepileptic drug (AED) treatment, there have recently been several reports that approximately 15-20% of patients with JME are resistant to such treatment, despite an accurate diagnosis and choice of AEDs. In this study, we sought to identify risk factors that may lead to treatment resistance in patients with JME. Subjects and Methods: The subjects were 47 patients meeting the criteria of JME, and had been followed up for over 2 years. We retrospectively analyzed the response to treatment, and classified them into 3 groups: group 1 consisting of fully controlled cases (N=33), group 2 of a true resistant case (N=1), and group 3 of pseudoresistant cases (N=13). The epileptic seizures in group 2 were difficult to control despite various AED treatments from the onset of epilepsy. Group 3 cases showed a recurrence of seizures despite excellent initial responses to AEDs. Among the group 3 cases, 4 patients showed a low compliance with AEDs because of poor recognition of their epilepsy, while the remaining 9 patients had serious psychosocial factors potentially aggravating the seizures. Conclusion: Approximately 30% of patients with JME experienced a recurrence of seizures despite an appropriate choice of AEDs. Most of them were categorized into refractory JME due to various psychosocial factors. Our results suggest that seizure control and the quality of life in this group are improved by education, psychological treatment, and favorable life-styles.
We report a 70-year-old man, who developed painful involuntary muscle contraction of the left leg after the lumbar discectomy, which exacerbated after a vertebral fracture of Th12. This involuntary movement was accompanied with the abnormal position of left leg simulating triple flexion response, and was induced by active or passive movement of his left knee and foot joints. Several drugs including benzodiazepines and dantrolene were ineffective, although treatment with baclofen or carbamazepine was effective. These findings suggest that hyperexcitability of the anterior horn cells following the disturbance of spinal inhibitory interneurons was involved. Electophysiological studies suggested the disturbance of left lumber nerve roots. The spinal root blocks from L3 to S1 were performed, after which the painful involuntary muscle spasm was resolved. The lumbar sympathetic ganglia block was also effective; suggesting that abnormal afferent neuronal input to spinal cord was caused by the nerve root trauma which triggered the formation of secondary abnormal network in the spine. Lumbar sympathetic ganglia block should be recommended to a therapeutic option for the refractory painful muscle spasm of the leg.
A 38-year-old man gradually developed gait instability, dysarthria, and dysphagia over two months associated with an elevated blood pressure after starting hemodialysis therapy for diabetic nephropathy. Brain MRI studies indicated vasogenic edema in the brainstem, extending from the lower midbrain to the upper medulla oblongata. The patient's high blood pressure was refractory to treatment, and his neurological disabilities and MRI abnormalities progressed. FDG-PET, MR spectroscopy, and cerebrospinal fluid studies did not suggest neoplastic pathologies. The patient was diagnosed with a brainstem variant of reversible posterior leukoencephalopathy syndrome, and received three courses of steroid pulse therapy. After the pulse therapy, the clinical manifestations and MR findings improved. By maintaining strict management of blood pressure and body water balance during hemodialysis, he did not experience any further clinical exacerbation, and the lesion on MR images continued to regress. Ten months after the pulse therapy, T1-weighted images showed slightly hyperintense signal. This case suggests that reversible posterior leukoencephalopathy syndrome (RPLS) may take a chronic clinical course without acute onset.
We present two patients (case 1: a 78-year-old right-handed woman, case 2: a 66-year-old right-handed woman) with suspected transient global amnesia. Both patients showed sudden onset amnesia that appeared to resolve within 24 hours, and they showed no abnormal findings on electroencephalography or single photon emission tomography. However, the results of the Wechsler Memory Scale-Revised (WMS-R) on the ninth (case2) or tenth day (case1) after the onset indicated memory impairment. And diffusion-weighted magnetic resonance imaging (DWI) revealed a unilateral high-intensity area in the hippocampus, leading to the diagnosis of hippocampal infarction in both patients. Case 1 had a left hippocampal lesion and exhibited impairment of predominantly verbal memory, while case 2 had a right hippocampal lesion and demonstrated impairment of predominantly visual memory. When evaluating a patient with suspected transient global amnesia, imaging of the hippocampus and tests on memory function after an appropriate interval from the onset are often helpful in establishing a correct diagnosis.
A 75-year-old woman developed loss of vision and decreased ocular motility in all directions. She exhibited a left orbital apex syndrome, accompanied by sphenoiditis and hypertrophic pachymeningitis. Voriconazole treatment was initiated on the basis of clinical suspicion, although use of the serum β-D glucan had negative results and a biopsy was not performed. Five days later, the left eye movements started to improve, and at that time the use of the serum aspergillus galactomannan antigen proved to have positive results. Six months later, the patient was neurologically intact and stable, except for a lack of visual acuity in counting fingers. Earlier prognoses of invasive sino-orbital aspergillosis were dismal, especially when corticosteroid therapy was done before diagnosis. This case suggests the usefulness of antifungal agents during the diagnostic procedure even when localized invasive aspergillosis is not ruled out.
A 30-year-old man with intractable headache and high fever came to the emergency room. He presented skin eruption, aphtha, drowsiness (E3, V4, M6 /GCS), meningeal sign, hyperreflexia, and bilateral papilloedema. He also showed pleocytosis (1,066/μl, polymorphonuclear cells predominantly) and pressure increase in CSF. WBC count and CRP were elevated. Two years before, he had meningeal sign, cervical myelitis, skin eruption, uveitis, and aphtha, and was diagnosed as Neuro-Behçet (NBD) disease. HLA-B52 and B55 were positive. We suspected recurrent NBD, but we couldn't exclude bacterial meningitis because of his symptoms and cell-pattern in CSF. We treated him with intravenous methylpredonisolone and antibiotics (MEPM 6g/day and VCM 2g/day). His symptoms dramatically improved within a few days. The rapid improvement might be attributed to the steroids. Furthermore, MRI FLAIR images showed multiple small high lesions in the brainstem, hemispheres, subcortical area, putamen and left cerebellar hemisphere. Serum procalcitonin was not increased. We diagnosed the recurrence of NBD retrospectively. Procalcitonin may be a useful marker for discrimination between meningitis due to NBD and septic meningitis.