Trigeminal root entry zone infarct presenting as isolated trigeminal sensory neuropathy.
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Biomedical subjects
Publications and source records attributed to T Fukutake.
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OBJECT: To study the effects of the intravenous administration of methylcobalamin, an analogue of vitamin B12, for uremic or uremic-diabetic polyneuropathy in patients who are receiving maintenance hemodialysis. An ultra-high dose of vitamin B12 has been reported to promote peripheral nerve regeneration in experimental neuropathy. METHODS: Nine patients received a 500 microg methylcobalamin injection 3 times a week for 6 months. The effects were evaluated using neuropathic pain grading and a nerve conduction study. RESULTS: Serum concentrations of vitamin B12 were ultra-high during treatment due to the lack of urinary excretion. After 6 months of treatment, the patients' pain or paresthesia had lessened, and the ulnar motor and median sensory nerve conduction velocities showed significant improvement. There were no side effects. CONCLUSION: Intravenous methycobalamin treatment is a safe and potentially beneficial therapy for neuropathy in chronic hemodialysis patients.
The clinical features of a probably autosomal recessive syndrome ("CARASIL"), yet to be confined in Japan and characterized by prematurity of vascular dementia, alopecia and spondylosis deformans are reviewed through comparison with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), which has been reported in Europe and North America, and recently in Japan. These two syndromes have many common features, such as familiality, encephalopathy of Binswanger type, and absence of vascular risk factors. There exists, however, a number of differences as follows: (1) Onset of encephalopathy is 32 years of age in "CARASIL" vs. 45 in CADASIL. (2) Male to female ratio is 3.2: 1 vs. 2:1.(3) Two thirds of "CARASIL" patients show stroke and/or stepwise deterioration, while almost all CADASIL patients have stroke. (4) Associated psychiatric features are euphoria, emotional lability and loss of spontaneity vs. severe mood disorders. (5) Migraine is a cardinal feature of CADASIL and vasospasm may occur during cerebral angiography. (6) White matter lesions on MRI are diffuse and homogeneous vs. punctuated and nodular. The latter four differences may mirror the difference in the pathology of arteriopathies. "CARASIL" is clearly different from CADASIL and reflect a second genetic condition with a seemingly direct effect upon the cerebral vasculature.
We report a case of adrenomyeloneuropathy (AMN) in which serial urodynamic studies showed neurogenic bladder dysfunction. The patient was in good health until the age of 12, when he began to lose his hair. At age 25 he started to have urinary urgency, difficulty in voiding, occasional fecal incontinence, erectile impotence, and progressive gait disturbance. In his first admission to our hospital age 31, he was intelligent but childish. He showed diffuse baldness, spastic paraparesis, and disturbed vibratory sensation. Serum cortisol response to corticotropin (ACTH) was low and serum levels of very long chain fatty acids were increased. Nerve conduction studies and sural nerve biopsy showed the presence of peripheral neuropathy. These findings confirmed the diagnosis of AMN. The first urodynamic study showed residual urine volume of 50 ml, impaired bladder sensation, and detrusor hyperreflexia. At age 38 he needed diapers because he became apathetic and demented, and could no longer stand by himself. MRI disclosed high signal intensities in the bilateral cerebral white matter. The second urodynamic study showed residual urine volume of 200 ml and decreased bladder capacity with marked detrusor hyperreflexia. Demyelinating lesions of the peripheral nerve and white matter of the spinal cord and the cerebrum may be mainly responsible for the micturitional disturbance in our patient with AMN.
Thirty-one cases (26 patients) of clinically definite multiple sclerosis with predominant spinal cord involvement were studied retrospectively, focusing in particular on the results of sensory testings which were compared with the findings of spinal cord magnetic resonance imaging (MRI) and somatosensory evoked potentials (SEPs). Factor analysis of the sensory impairments showed two factors corresponding to superficial (pain and light-touch) and deep (position and 'thumb/big toe localizing') sensations. Vibratory sense depended on both factors, but more on deep sensations. Multiple regression analysis of the SEP abnormalities and sensory deficits showed that impaired deep sensations contributed to abnormal SEPs, but the results of clinical sensory testings and SEPs were dissociated in 31% of the extremities examined. The MRI sagittal images correlated well with the levels of sensory impairments, whereas the axial images showed a poor relationship to the extent of sensory impairments. We concluded that the use of SEPs as well as MRI is not always superior to such clinical sensory tests as the 'thumb/big toe localizing tests'.
Micturitional disturbance is rarely mentioned in human herpetic brainstem encephalitis although the pontine tegmentum, called the pontine micturition centre, seems to regulate the lower urinary tract in experimental animals. The case of a 45 year old man, who developed subacute coma and hiccup-like dysrhythmic breathing, and needed assisted ventilation is reported. Examination of CSF showed mononuclear pleocytosis and antibody against herpes simplex virus type 1, but the opening pressure was 90 cm H2O. Brain CT showed brain swelling, predominantly in the posterior fossa, and bilateral subdural effusion. Herpetic brainstem encephalitis was diagnosed, and he received 900 mg/day vidarabine. On regaining consciousness, he had left trochlear nerve palsy, left corectopia, ageusia, and urinary retention. Brain MRI showed right side dominant, bilateral pontine segmental lesions extending slightly to the midbrain and medulla. After two weeks he was able to urinate but showed nocturnal urinary frequency, urinary incontinence, and voiding difficulty. Urodynamic studies showed a residual urine volume of 350 ml and detrusor hyporeflexia on voiding. Micturitional disturbance gradually disappeared together with the neurological signs. The bilateral pontine tegmental lesions in this patient are similar to those in previous findings on brainstem strokes, evidence of the presence of a pontine micturition centre in humans.
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Although the medial geniculate body (MGB) is a well-known integration center for the central auditory pathways, auditory symptoms caused by MGB damage have not been described. We report the appearance of the auditory illusions of hyperacusis and palinacousis in a 49-year-old man without previous psychiatric or epileptic illness after a small hemorrhagic infarction located mainly in the right MGB.
Since the introduction of the tracheoesophageal fistula (TEF) technique for restoration of voice in 1983, we performed voice restoration using primary tracheoesophageal fistula in 83 patients undergoing laryngectomy. The success rate for voice restoration with TEF was 80.6% (good voice; 69.8%, fair voice; 10.8%). Thirty-one patients out of 51 patients who have been followed for over 3 years had 34 problems with TEF (15 granulations of TEF, 7 difficulties in insertion of voice prostheses, 4 stenoses of the tracheal stoma, 5 other reasons). However, most patients could use TEF by following appropriate procedures. Our results suggest that the Blom Singer TEF procedure is a safe, simple and effective means of alaryngeal communication.
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A 37-year-old woman with a 5-year history of multiple sclerosis is reported. She began having recurrent syncope even in the sitting position; other neurological features included hiccup, faciooro-lingual flushing and clumsiness of the hands. She had alternating Horner's syndrome, mild hypoalgesia of the right face, exaggerated deep tendon reflexes of the upper extremities, decreased deep sensation and ataxia of the upper extremities, and incomplete transverse myelopathy with a T4 sensory level. Head-up tilt testing confirmed orthostatic hypotension with relative preservation of the heart rate increase. Magnetic resonance imaging indicated abnormal intensities in the paramedian tegmentum and base of the medulla, which may have been additionally responsible for orthostatic hypotension. Steroid pulse therapy and L-threo-3,4-dihydroxyphenylserine caused regression of brainstem signs and reduced syncopal attacks.
A 30-year-old woman with Marfan's syndrome had chronic intractable headaches and spontaneous intracranial hypotension. The pain was concentrated over the occipitonuchal region, had elements of both migraine and tension headache, and was often aggravated by postural change. Myelography showed multiple, large, lumbosacral arachnoid diverticula. Radioisotope cisternography revealed a halolike accumulation in the lumbosacral region and rapid uptake of isotope in the urinary bladder, indicating cerebrospinal fluid leakage. Epidural blood patching brought immediate relief from the positional headaches. We concluded that patients with Marfan's syndrome and undifferentiated chronic headaches should be radiologically evaluated for spinal meningeal defects.
The virus-associated hemophagocytic syndrome (VAHS) is a lymphoproliferative disorder induced by viral infection. The peripheral nervous system is rarely involved in this syndrome and, when reported, the involvement has been axonal in nature. This paper presents a case of Guillain-Barré syndrome accompanied by VAHS subsequent to Epstein-Barr virus infection. The patient's condition improved following treatment with intravenous immunoglobulin infusion and high-dose corticosteroids.
To assess the prevalences and neurological symptoms of destructive spondyloarthropathy (DSA) and other cervical spinal diseases in long-term hemodialysis, a questionnaire survey of the medical histories and subjective sensory-motor symptoms was taken on 191 patients undergoing dialysis for 0.2-23 years (mean 8.6 +/- 6.3) with a mean age of 56.6 +/- 11.8 years (range 23-86). Furthermore, plain radiographic examinations of the cervical spine were carried out in 90% of the patients, and neurologic examinations were also performed on 29 patients. DSA was diagnosed in 18 cases (10%): moderate narrowing of the intervertebral space (NIS-I) in 8, severe narrowing (NIS-II) in 18, ossification of posterior longitudinal ligaments (OPLL) in 6, cervical spondylosis (CS) in 12 and other abnormalities in 6; 104 cases were normal. NIS-I was considered to reflect an early stage of DSA. The age of patients with DSA (mean 61.5 +/- 10.2 years), as well as with CS, was significantly higher than the normal group (52.2 +/- 10.6; p < 0.001), but younger than CS (68.5 +/- 10.0). The duration of dialysis in DSA patients (mean 12.1 +/- 6.0 years) was the longest and significantly longer than normal (7.7 +/- 6.5; p < 0.01) and (:S patients (3.8 +/- 3.0; p < 0.001). Through comparative evaluations of prior histories of the diseases, such as diabetes mellitus, carpal tunnel syndrome, amyloid osteoarthropathy (AOA) and parathyroidectomy, along with large calcification of the nuchal soft tissues on plain films, it was found that DSA, in conjunction with NIS-I, correlated with only AOA (p < 0.05). Subjective sensory symptoms, which consisted chiefly of arthralgic type followed by polyneuritic type, were noted in 60% of all patients and 56% of DSA patients. No significant differences were evident between any two particular groups. The score of subjective motor symptoms of the lower extremities in 5 patients with myelopathy was significantly higher than that in 5 other patients without neurological disorders (p < 0.05). Thirty-eight percent of DSA patients showed myelopathy; the frequency was higher than those of previous similar studies (0 = 5%). In conclusion, this study demonstrated that 1) DSA occurred in 10% of dialysis patients, which was also evident in previous reports (9 = 10%); this prevalence was higher than those of CS or OPLL, 2) DSA correlated with the duration of dialysis and AOA, 3) subjective motor symptoms of the lower extremities could indicate presence of myelopathy whereas subjective sensory symptoms contributed little to differential diagnosis, and 4) a careful neurologic examination can more frequently disclose myelopathy in patients with DSA than so far believed.
Erythromelalgia is a rare disorder characterized by burning pain of the extremities associated with red discoloration and increased temperature of the skin. We observed the effects of several drugs on two patients with primary erythromelalgia. Anti-inflammatory drugs such as aspirin had no effects for their symptoms. The alpha-adrenergic agonist midodrine slightly reduced skin temperature and redness of the extremities, and minimally reduced the pain in one patient. The partial alpha-adrenergic agonist and 5-hydroxytryptamine (5-HT) agonist/antagonist dihydroergotamine transiently reduced skin temperature and pain to some degree in one patient. The 5-HT and histamine antagonist cyproheptadine effectively relieved the burning pain and increased skin temperature.
Two Japanese male siblings, aged 68 and 59 years, affected by late-onset progressive ataxia distinguished by extensive sensory and mild autonomic disturbances are described. They had global thermoanalgesia, positive Romberg signs, sensorineural deafness, canal paresis and ageusia. Their autonomic disturbances consisted of absence of overflow tears with usual stimuli, dysphagia, blood pressure and vasomotor instability, diarrhoea/constipation, and urinary frequency. Sensory nerve action potentials were completely absent, whereas motor conduction velocity was slightly reduced only in the lower extremities. Sural nerve biopsy on the younger brother demonstrated a marked loss of myelinated fibres and a reduction in the number of unmyelinated axons. Tongue histology revealed absence of fungiform papillae and taste buds. Autonomic function tests showed widespread but mild sympathetic and parasympathetic failures. Neuro-imaging studies revealed atrophy of the spinal cord, cerebellum, brainstem and corpus callosum, and enlargement of the lateral, third and fourth ventricles. These siblings represent a previously unrecognized variant of late-onset hereditary spinocerebellar degeneration with global thermoanalgesia and absence of fungiform papillae on the tongue.