Cerebral white matter lesions in acute motor axonal neuropathy.
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Acute Disease↗
Biomedical subjects
I Funakawa
Publications and source records attributed to I Funakawa.
[A case of zoster sine herpete with involvement of the unilateral IX, X and XI cranial and upper cervical nerves].
We describe a case of unilateral IX, X and XI cranial and upper cervical nerve palsies involving zoster sine herpete (ZSH). A 63-year-old man experienced nausea, loss of appetite and general fatigue. On 4 days of illness, dysphagia, dysarthria and difficulty in elevation of his right arm appeared. Neurological examination showed the right curtain sign, a nasal voice and a decreased right gag reflex. He could hardly elevate his right arm laterally. Needle electromyography revealed positive sharp waves in his right trapezius muscle. Although no skin lesion was detected, anti-varicella-zoster virus antibodies were positive in both serum and cerebrospinal fluid. Acyclovir and a steroid were ineffective for these symptoms. Although case reports of unilateral IX, X and XI cranial nerve palsies with ZSH is very rare, ZSH should be kept in mind in the differential diagnosis of multiple cranial nerve palsies.
Accessory Nerve Diseases↗
[A case of Bickerstaff's brainstem encephalitis during pregnancy].
Here we present a case of 23-year-old woman with Bickerstaff's brainstem encephalitis (BBE) during 13 weeks of pregnancy. After symptoms of the upper respiratory tract infection, she developed somnolence, marked disturbance of extraocular eye movements, cerebellar ataxia, hyperreflexia and spasticity of the lower limbs. An electroencephalogram revealed slow waves and brainstem auditory evoked potential disclosed prolongation of III-V interpeak intervals. Serum IgG anti-GQ1b antibody was detected. Plasma exchanges (PE) were performed four times. Although no patient with BBE during pregnancy has ever been reported, in the literatures the pregnant patients with Guillain-Barré syndrome had four sessions of plasmapheresis with safe. Neurological symptoms gradually improved after the PE. No serious adverse effects were noted. Although no definite therapy for BBE has been established, PE may be one of the selections even if the patients is pregnant.
Adult↗
Intractable hiccups and syncope in multiple sclerosis.
A patient with multiple sclerosis (MS) who developed intractable hiccups and syncope due to a cervical cord lesion is reported. Previous case reports of intractable hiccups occurring in MS have all been located in the medulla oblongata. Our patient is the first case with the responsible lesion in the cervical cord. The pathophysiological mechanism of the syncopal attacks in this case were thought to be same as that of cough syncope.
Cervical Vertebrae↗
Periodic lateralized epileptiform discharges in mitochondrial encephalomyopathy.
Periodic lateralized epileptiform discharges (PLEDs) were transiently recorded during the acute phase of cerebral stroke-like episodes in three patients with mitochondrial encephalomyopathy (MEM). One of the patients developed stroke-like episodes three times, always accompanied by PLEDs. These discharges were recorded from the hemisphere responsible for the stroke-like episodes or from the more severely affected hemisphere when bilateral hemispheres were affected. Although PLEDs have been rarely reported in MEM, this disease should be considered as a differential diagnosis of cerebral infarction with PLEDs.
Adult↗
[Adult pneumococcal meningitis complicated by cerebral infarction: a case report].
We describe a 36-year-old man with pneumococcal meningitis who suddenly showed a left central type facial palsy and left hemiparesis. Magnetic resonance imaging (MRI) revealed a low intensity area in the posterior limb and genu of the right internal capsule to caudate nucleus in T1-weighted images and a high intensity area in T2-weighted images. We surmised vasculitis as the cause of cerebral infarction, because steroid treatment was effective.
Adult↗
Cerebellar ataxia in patients with Leber's hereditary optic neuropathy.
We report the cases of a mother and son with Leber's hereditary optic neuropathy (LHON), where a point mutation of mitochondria DNA from guanine to adenine on nucleotide position 11778 was verified. Both also had cerebellar ataxia and dysarthria and in both cases cerebellar atrophies were detected by computed tomography or magnetic resonance imaging. It was not possible to elucidate the relationship between LHON and the cerebellar atrophy, but it should be kept in mind that various neurological complications may occur in LHON.
Adult↗
[Serial 123I-IMP SPECT in a case of cryptococcal meningoencephalitis].
We performed serial brain perfusion SPECT studies using 123I-IMP in the course of cryptococcal meningoencephalitis. Increased perfusion was detected in the cerebellum coinciding with the appearance of the abnormality on MRI. The perfusion in the lesion was then decreased as the abnormality on MRI disappeared. Cryptococcal meningoencephalitis should be also taken in the differential diagnosis when the inflammatory brain lesion shows increased perfusion.
Aged↗
[A case of MELAS associated with prosopagnosia, topographical disorientation and PLED].
We report a case of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) associated with prosopagnosia, topographical disorientation, and periodic lateralized epileptiform discharge (PLED) on electroencephalography (EEG) in a 23-year-old right-handed man. The first MELAS attack occurred on March 1, 1991, while the patient was drinking. Magnetic resonance imaging (MRI) revealed a lesion of abnormal intensity in the left occipital lobe. The second attack occurred on October 1, 1991. This time, the major symptoms were visual loss of acute onset, nausea, and vomiting. EEG examination showed transient PLED. MRI revealed a new area of abnormal intensity in the right occipital lobe, lingual gyrus, fusiform gyrus and the posterior part of the parahippocampal gyrus. During the clinical course of the patient, prosopagnosia and topographical disorientation appeared. There have been few reports of MELAS associated with prosopagnosia, topographical disorientation, and PLED. However, MELAS attacks tend to occur in the cortex of the occipital lobe. We therefore believe that these neuropsychological symptoms and PLED are likely to be associated with MELAS.
Adult↗
[Relationship between thermal threshold and population of peripheral nerve fibers].
The relationship skin thermal threshold (TT) and density of both myelinated and unmyelinated fibers were investigated in 12 patients with various peripheral nerve diseases including Crow-Fukase syndrome, carcinomatous neuropathy and Charcot-Marie-Tooth disease. The TT was measured at a site above the medial tibial condyle using a thermal threshold tester. The TT was defined as the threshold at which a slight rise or fall in temperature could be detected. The size of the skin stimulation area was 13.5 cm2, the temperature change rate was 1 degrees C/sec, and the basal temperature was set at 34 degrees C. A sural nerve biopsy was performed and an image processor (IBAS) was used to measure the nerve fiber density. The TT in the patients with peripheral nerve disease was found to be elevated when compared with that of the 74 healthy controls (mean age of 38.1 +/- 13.3 years). Elevation of the TT has an intimate relation to damage of the small myelinated nerve fibers. In conclusion, unmyelinated fibers may play a relatively minor role in conveying thermal sensation.
Adult↗
[Magnetic resonance imaging of spinal cord lesions in 22 multiple sclerosis patients].
We reviewed MRI findings in 22 patients (37 cases) with clinically diagnosed multiple sclerosis (MS) with spinal cord lesions. The spinal cord lesions were detected in 17 (46%) of these 37 cases on MRI. The cervical cord lesions were more detectable than other spinal cord lesions. At the thoracic level, the upper lesions were more detectable than the lower ones. In this study, no correlation was found between the disease duration, the rate of functional disturbance and the detectable rate of spinal cord lesions. The characteristic findings of the lesions were swelling and the enhancement effect of Gd-DTPA in the patient group with a disease duration of less than three years, and atrophic change in the patient group with a disease duration of greater than seven years. The period of the enhancement effect of Gd-DTPA varied in each case, and it may reflect the clinical course. Syrinx-like lesions were found in four cases. In one of them, atrophic change was found in the same region six months after the follow up study. Although the precise reason for the syrinx-like lesion was unclear, a relationship between syrinx-like lesion and atrophy of the spinal cord was suggested. The MRI findings of the spinal cord lesions in MS varied in each case and in each stage of the disease.
Adolescent↗
Intractable hiccups and sleep apnea syndrome in multiple sclerosis: report of two cases.
Two cases of multiple sclerosis associated with intractable hiccups (IH) and sleep apnea syndrome (SAS) are reported. Lesions were detected in the tegmentum of the medulla oblongata by magnetic resonance imaging. In one case, high dose methylprednisolone was remarkably effective for the IH. For the SAS, amitriptyline was effective in one case. The IH and SAS are thought to be important symptoms when a lesion occurs in the tegmentum of the medulla oblongata, including the paramedian and lateral reticular formations. If IH appears in conjunction with a lesion in the tegmentum of the medulla oblongata, one must be vigilant for the development of SAS.
Amitriptyline↗
[A case of cryptococcal meningitis in an HTLV-1 carrier].
We report a case of 73-year-old male HTLV-I carrier with cryptococcal meningitis. The patient, who was born in Taiwan, has raised golden pheasants for ten years and bantams for five years. Antibody to HIV was negative. Flow cytometric study of the peripheral lymphocytes showed reduced CD4+CD45RA+ (naive cells) and increased CD4+CD45RO+ (memory cells), CD3+CD25+ and CD3+ HLA-DR(DR)+ cells. Lymphocyte responses to phytohemagglutinin and concanavalin A were depressed. Cerebrospinal fluid (CSF) cells and serum and CSF antigen to cryptococcal neoformans were decreased by therapy with fluconazole and flucytosine. Although the naive, memory and CD3+DR+ cell abnormalities showed no change, the CD45RA/CD45RO ratio and CD3+CD25+ level tended to improve. Opportunistic infections such as cryptococcal meningitis may be induced by severe decreases in naive cells and increases in memory cells in HTLV-I carriers.
Aged↗
[A case of multiple sclerosis with intractable hiccups and sleep apnea syndrome].
A 48-year-old female with multiple sclerosis (MS) accompanied by intractable hiccups of over one month' duration and the sleep apnea syndrome was reported. This MS patient had been well controlled until September 16, 1991 when she experienced nausea, vomiting and hiccups. The patient was admitted to Kawasaki Medical School Hospital on October 9, 1991. A physical examination revealed intractable hiccups. T1-weighted MRI showed a low and T2-weighted image disclosed a high signal intensity area in the tegmentum of the medulla oblongata. The intractable hiccups and vomiting improved with intravenous high dose methylprednisolone injection therapy. The following day, she complained of insomnia and her family observed severe snoring and apnea during the night. These symptoms and the results of a breathing monitor were compatible with the sleep apnea syndrome. These symptoms disappeared following the administration of amitriptyline. There have been few reports of the combination of intractable hiccups and the sleep apnea syndrome in MS. The MRI findings suggest that the causative lesion of these symptoms is in the tegmentum of the medulla oblongata.
Amitriptyline↗
[A case of Sotos syndrome associated with peripheral nerve involvements].
A case of the Sotos syndrome associated with peripheral nerve involvements was reported. A 52-year-old male was admitted to Kawasaki Medical School Hospital because of gait disturbance, muscle atrophy, and weakness in both hands. This case was diagnosed as the Sotos syndrome based on the following symptoms and findings, acromegaloid features, hypertrophic changes in the hands and feet, a history of epileptic episodes, a low IQ, a normal growth hormone value, and no tumor lesion in the pituitary gland. Radiological examination disclosed a cauliflower-like appearance of the finger tips and thickness of the heel pads. Brain CT and MRI revealed diffuse mild brain atrophy. An electroencephalogram showed diffuse theta waves with sharp waves in the right parietal region. A needle electromyogram revealed neurogenic change in both upper and lower limbs. A nerve conduction study disclosed the carpal tunnel syndrome and cubital tunnel syndrome. These findings suggest that, as in the case of acromegaly, entrapment neuropathy and peripheral neuropathy can also be induced in the Sotos syndrome.
Brain Diseases↗
[A case of bilateral blepharospasm responsive to edrophonium].
A case of bilateral blepharospasm who registered the efficacy of edrophonium was reported. The case is a 49-year-old female. She had been in good health until January, 1991 when she complained of difficulty in opening her eyes while driving. Thereafter the condition progressed to such a degree that she was unable to experience a comfortable life. Her blinking rate did not changed. The symptoms were triggered by stress or some physical action, such as walking or driving. They were attenuated by taking a bath, sleep or sedation. The severity of the symptoms varied during the day and from day to day. Neurological examination revealed bilateral spasms of the orbicular oculi muscles, and occasionally of the orbicular oris muscles, sternocleidmastoid muscles and the perinasal regions. Neither orolingual dyskinesia nor other involuntary movements were detected. Surface electromyography (EMG) disclosed tonic discharges mainly from the orbicular oculi muscles. The abnormal spasm disappeared with the injection of edrophonium chloride. The test for the serum antiacetylcholine receptor antibody was negative and a repetitive stimulation EMG showed no waning phenomenon. No thymoma or thymus abnormalities were detected by pneumomediastinography. A needle EMG revealed neurogenic change in the distal portion of the limbs. A single fiber EMG showed elongation of the jitter value and the blocking phenomenon. Although distigmine bromide was ineffective against the spasm, pyridostigmine bromide and the local injection of botulinum toxin were very effective.(ABSTRACT TRUNCATED AT 250 WORDS)
Blepharospasm↗
Single photon emission computed tomography (SPECT) in patients with periodic lateralized epileptiform discharges (PLEDs).
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Aged↗