Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “REFLEX, TENDON”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,297 records · Page 72Linked to original sources
PubMed2003

[A case of subacute combined degeneration with normal serum vitamin B12 level].

A 40-year-old woman was admitted to our hospital because of pancytopenia with megaloblastic anemia. Two months later she complained of rapidly progressive gait disturbance and numbness in the distal part of limbs. She also told that her hair had turned totally gray in the third decade. Neurologically, mental state, cranial nerves and cerebellar functions were normal. Superficial sensations were impaired below the lower thoracic level and deep sensations were completely lost in the lower limbs. Moderate weakness was found in the lower limbs, symmetrically. Deep tendon reflexes were diminished in the upper limbs and absent in the lower limbs. Babinski's reflex was positive bilaterally. MR images of the spinal cord showed hyperintensity in the posterior column below the thoracic cord. Although the serum level of vitamin B12 was within normal range, serum homocysteine level was elevated markedly. Under the diagnosis of subacute combined degeneration (SCD) due to possible vitamin B12 deficiency, the treatment with intravenous injections of 500 micrograms/day of mecobalamin was undertaken. Muscle strength and sensory impairment improved progressively and she became able to walk with a cane. The coloration of her gray hair was also noted. After treatment, pancytopenia and megaloblastic anemia also markedly improved. Vitamin B12 became high in serum concentration and the serum level of homocysteine became normal. These clinical and laboratory findings support the diagnosis of SCD with normal serum level of vitamin B12 in our case, suggesting that the level of vitamin B12 in serum does not always correlate with that in tissue and, therefore, SCD should not be excluded just only by the reason of normal serum vitamin B12 level.

Adult↗
PubMed2003

[Cheiromegaly and podomegaly associated with primary progressive multiple sclerosis: a case report].

Cheiromegaly is an extremely rare syndrome characterized by unilateral hand hypertrophy with hyperhydrosis. Unilateral foot hypertrophy has been described as podomegaly. Cheiromegaly/podomegaly is usually seen in patients with syringomyelia. Although the underlying pathophysiology remains unknown, it is speculated that chronic cervical cord injury including central descending sympathetic tract is essential for development of this syndrome. We report a cheiromegaly/podomegaly in a case with primary progressive multiple sclerosis. This 29-year-old woman developed cheiromegaly and podomegaly in her right hand and foot associated with chronic progressive cervical myelopathy. At age two, she developed transient paraparesis with full recovery, suggesting her symptomatic onset of her longstanding illness. At age 10, she had left leg weakness, and gradually worsened. On admission at age 29, she had muscular weakness in her left upper and both lower extremities. Sensory impairment was seen below her 5th cervical and 6th thoracic dermatome level. Her deep tendon reflexes were exaggerated in all extremities with bilateral Babinski's signs. MR imaging showed multiple plaques in the white matter of the brain associated with cervical spinal cord lesions. Taken together with the presence of oligoclonal band IgG in the cerebrospinal fluid, this patient met the criteria of multiple sclerosis. This is the first report presenting cheiromegaly/podomegaly not associated with syringomyelia, but with multiple sclerosis. It is suggested that cheiromegaly/podomegaly in present case may be caused by chronic cervical cord injury affecting descending central sympathetic tract associated with primary progressive multiple sclerosis.

Acromegaly↗
PubMed1992

The relation between motor evoked potential and clinical motor status in stroke patients.

Forty-one stroke patients and 41 normal controls of matched age, sex and body height were studied by magnetic stimulation of the motor cortex and lower cervical area. The cortical and cervical motor evoked potentials (MEPs) were detected from the abductor pollicis brevis muscle. The amplitudes and latencies of cortical MEPs, central conduction time (CCT), and interside difference of bilateral CCT (DCCT) in stroke patients showed significant difference in comparison with normal controls. The degree of motor weakness was statistically correlated with the severity of cortical MEP findings, but not with the deep tendon reflex and Babinski sign. The abnormal rate of cortical MEP was higher in patients with hemorrhage than in those with infarction. Six out of 17 patients with hemorrhage had prolonged CCT or DCCT, but only 3 out of 24 patients with infarction had the same findings. The cortical MEP findings in putaminal hemorrhage were more severe than in thalamic hemorrhage. In instances of infarction involving total middle cerebral artery territory (cortical and subcortical areas), the cortical MEP was absent in all cases (4/4). It was concluded that cortical MEP findings in stroke patients examined by magnetic stimulation showed good correlation both with the lesion radiologically and the motor deficit clinically. This is an acceptable method for quantitative evaluation of motor system function in stroke.

Adult↗
PubMedDate not supplied

[Psychiatric manifestations of vitamin B12 deficiency: a case report].

Psychiatric manifestations are frequently associated with pernicious anemia including depression, mania, psychosis, dementia. We report a case of a patient with vitamin B12 deficiency, who has presented severe depression with delusion and Capgras' syndrome, delusion with lability of mood and hypomania successively, during a period of two Months. Case report - Mme V., a 64-Year-old woman, was admitted to the hospital because of confusion. She had no history of psychiatric problems. She had history of diabetes, hypertension and femoral prosthesis. The red blood count revealed a normocytosis with anemia (hemoglobin=11,4 g/dl). At admission she was uncooperative, disoriented in time and presented memory and attention impairment and sleep disorders. She seemed sad and older than her real age. Facial expression and spontaneous movements were reduced, her speech and movements were very slow. She had depressed mood, guilt complex, incurability and devaluation impressions. She had a Capgras' syndrome and delusion of persecution. Her neurologic examination, cerebral scanner and EEG were postponed because of uncooperation. Further investigations confirmed anemia (hemoglobin=11,4 g/dl) and revealed vitamin B12 deficiency (52 pmol/l) and normal folate level. Antibodies to parietal cells were positive in the serum and antibodies to intrinsic factor were negative. An iron deficiency was associated (serum iron=7 micromol/l; serum ferritin concentration=24 mg/l; serum transferrin concentration=3,16 g/l). This association explained normocytocis anemia. Thyroid function, hepatic and renal tests, glycemia, TP, TCA, VS, VDRL-TPHA were normal. Vitamin B12 replacement therapy was started with hydroxycobalamin 1 000 ng/day im for 10 days and iron replacement therapy. Her mental state improved dramatically within a few days. After one week of treatment the only remaining symptoms were lability of mood, delusion of persecution, Capgras' syndrome but disappeared totally 9 days after the beginning of the treatment. A neurologic examination was possible because of cooperation. All the tendon reflexes of inferior members were absent. The plantars were in flexion and there was a left inferior member hypoesthesia. The cerebral scan and EEG were normal. Fundic biopsy, realized by fibroscopy, revealed fundic atrophia and intestinal metaplasia compatible with Biermers' disease. The iron deficiency exploration concluded diet deficiency. Mme V. appeared euphoric, her speech was very rapid with play on words and overactivity. This hypomania state totally disappeared 3 days after. Six Months after her hospitalisation, she presented an hypothyroidism (TSH=3,780; T3=1,35; T4=1,08). A thyroid hormones replacement was started and she continued to receive Monthly B12 replacement. Discussion - This case report illustrates psychiatric manifestations of Biermers' disease. The clinical arguments in favour are: white woman, more than 60 Years old, no history of psychiatric problems, atypical symptoms (confusional state with psychiatric symptoms), fluctuation of symptoms (severe depression with confusional state, delusion of persecution and Capgras' syndrome; delusion with lability of mood and hypomania), dramatic improvement after 9 days of vitamin B12 replacement therapy. The biological arguments are: anemia, vitamin B12 deficiency, normal folate level, atrophia and fundic metaplasia, positive antibodies to parietal cells in the serum, association between Biermers' disease and autoimmune disease (Haschimoto thyroidite). Psychiatric manifestations can occur in the presence of low serum B12 levels but in the absence of the other well recognized neurological and haematological abnormalities of pernicious anemia. Mental or psychological changes may precede haematological signs by Months or Years. They can be the initial symptoms or the only ones. Verbank et al. described the case of a patient with vitamin B12 deficiency in whom hypomania, paranoia and depression had been successively presented during a period of 5 Years before anemia have been developed. The case of Mme V. is similar in the succession of severe depression with delusion of persecution and Capgras' syndrome, delusion with lability of mood and hypomania, during a period of two Months. This report seems to be the first one of a sequence of several psychiatric states with pernicious anemia during a period of two Months with normocytosis anemia. To illustrate this illness we reviewed the literature regarding psychopathology associated with B12 deficiency. The most common psychiatric symptoms were depression, mania, psychotic symptoms, cognitive impairment and obsessive compulsive disorder. The neuropsychiatric severity by vitamin B12 deficiency and the therapeutic efficacy depends on the duration of signs and symptoms. Conclusion - We recommend consideration of B12 deficiency and serum B12 determinations in all the patients with organic mental disorders, atypical psychiatric symptoms and fluctuation of symptomatology. B12 levels should be evaluated with treatment resistant depressive disorders, dementia, psychosis or risk factors for malnutrition such as alcoholism or advancing age associated with neurological symptoms, anemia, malabsorption, gastrointestinal surgery, parasite infestation or strict vegetarian diet. In first intention, B12 deficiency should be researched by serum B12 determination (normal 200-950 pg/ml). Studies of methylmalonic acid and homocysteine showed that they are very sensitive functional indicators of cobalamin status especially when other evidence of cobalamin (B12) deficiency was equivocal. Measurement of methylmalonic acid (normal 73-271 nmol/l) and homocysteine (normal 5,4-13,9 micromol/l) should not replace the measurement of serum cobalamin.

Depression↗
PubMedDate not supplied

Neuropathology of the brain and spinal cord in human West Nile virus infection.

OBJECTIVE: To describe the histopathology of the brain and spinal cord in human West Nile virus (WNV) infection. MATERIALS AND METHODS: Single case report, including premortem clinical and laboratory findings, and autopsy. RESULTS: An 83-year-old female presented with acute confusion, high fevers, dysarthria and generalized subjective weakness, with decreased deep tendon reflexes and weakness on physical examination. Electromyography showed evidence of a sensorimotor axonal polyneuropathy of the right-sided extremities. She became ventilator-dependent and died after a 2-week ICU stay, following withdrawal of life support. WNV infection was confirmed premortem by detection of IgM antibodies from serum and CSF and postmortem by RT-PCR from brain tissue. Examination of the brain parenchyma showed scattered microglial aggregates accompanied by perivascular chronic inflammation. The leptomeninges showed focal lymphocytic infiltrates. Examination of the spinal cord showed lymphocytic infiltrates in nerve roots and within the cord proper, with focal microglial nodules and neuronophagia in the ventral horns. Special stains were negative for a demyelinating process. General autopsy revealed only emphysema and atelectasis. CONCLUSIONS: The findings in this case suggest direct viral infection of the spinal cord and nerve roots as the mechanism of the flaccid paralysis often observed in patients infected with WNV. Findings are reviewed in comparison with other reports of neuropathologic findings in human WNV infection.

Aged↗
PubMed2004

Neuromuscular paralysis in the intensive care unit.

OBJECTIVE: To determine the features, causes, risk factors and outcome of acquired neuromuscular paralysis in critically ill patients. METHODS: Retrospective review of all confirmed cases of acquired polyneuropathy and myopathy examined by our Neurology service in the Intensive Care Unit (ICU), at King Fahad National Guard Hospital, Riyadh, Kingdom of Saudi Arabia over a period of 5 years. All patients had comprehensive electrophysiological studies and one third had muscle and nerve biopsies. RESULTS: Thirty cases were included, 8 cases of polyneuropathy, 15 cases of myopathy and 7 cases of mixed neuropathy and myopathy. Absent deep tendon reflexes and absent sensory potential on nerve conduction studies were significantly suggestive of neuropathy. The level of creatine phosphokinase was not of great diagnostic value. Most polyneuropathy and myopathy cases had passed through a stormy ICU course with sepsis and multiorgan failure. The use of high doses of steroids was more associated with myopathy. Seven patients died in ICU, the others were discharged to the wards after a mean ventilation period of 40 days. One patient became chronic ventilator dependent. CONCLUSION: From this series and available literature, it seems that symptomatic myopathy is more frequent than polyneuropathy and some risk factors are common for both (sepsis and multiorgan failure) while the use of steroids is more associated with ICU myopathy. Treating sepsis and stopping corticosteroids results in the improvement of most of the cases.

Adolescent↗
PubMed1992

Intrathecal baclofen in hereditary spastic paraparesis.

Intrathecal baclofen has not been previously evaluated for the treatment of the disabling hypertonia associated with hereditary spastic paraparesis. Muscle tone and deep-tendon reflexes were evaluated in three patients with hereditary spastic paraparesis after a double-blind, cross-over bolus injection of intrathecal baclofen. Patients underwent placement of a subcutaneous pump for continuous infusion of intrathecal baclofen. Three months after implantation the muscle tone decreased 2.04 points (p less than .0001) and the reflex score decreased 2.25 points (p less than .001). Patients initially reported subjective weakness, but muscle testing revealed either an increase or no change in voluntary motor function. Baclofen doses of 60 to 264 micrograms per day were required for effective control of muscle tone and spasticity. Much of the disability in familial spastic paraparesis may be related to the loss of suprasegmental inhibition of spinal reflexes overwhelming the residual voluntary motor function.

Baclofen↗
PubMed2004

[Reversible MRI findings of posterior column of the spinal cord in a patient with acute autonomic and sensory neuropathy].

We report serial spinal MRI T2 findings in a patient with acute autonomic and sensory neuropathy (AASN). A 20-year-old woman was admitted to our hospital with progressive sensory disturbance in her extremities and orthostatic syncope after her symptoms of upper respiratory infection. Neurological examination demonstrated reduced tendon reflexes, hypalgesia, paresthesia, reduced position sensation in distal dominant extremities (predominant in lower legs) and wide variety of autonomic dysfunction (severe orthostatic hypotension, anhidrosis, urinary disturbance, coughing attack, constipation and appetite loss). She was diagnosed as having AASN. Although high dose intravenous immunoglobulin therapy successfully prevented the symptom progression, her sensory disturbance and autonomic dysfunction were prolonged and showed only slow improvement. Spinal MRI on acute phase was normal. On chronic phase (11 month after the onset), spinal MRI T2 weighted images demonstrated high intensity lesion in the posterior column successive from upper cervical to lower thoracic spinal cord. Those abnormal findings were attenuated in concordance with her symptom improvement and finally disappeared when she became to walk stably without assist.

Acute Disease↗
PubMed2004

[A case of HNPP presenting with late onset of symptoms, chronic progressive course and sensory impairment in face and trunk].

A 69-year-old man was admitted with a chief complaint of progressive muscle weakness and tingling sensation in the distal portion of all extremities since the age of 55. On neurological examination, sensory impairment of all modalities was noted in the right side of face, segmental areas of the right Th4-10 and the distal portion of four extremities. Symmetrical muscle weakness and atrophy was also found over the distal portion of all extremities. All deep tendon reflexes except biceps brachii reflexes were absent. Neurophysiological studies, however, rather indicated mononeuritis multiplex in this case. The biopsied specimen of the sural nerve showed a significant decrease in large myelinated fibers and many tomaculous changes. The gene analysis revealed deletion in the CMT 1A locus on chromosome 17p11.2, providing evidence for the diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP). The development of sensory impairment in face or thoracic nerves is quite rare in HNPP, indicating that there exists considerable phenotypic heterogeneity in the disease.

Aged↗
PubMed2004

[MELAS-like episodes in an adult case with cytochrome c oxidase deficiency].

A 30-year-old man was hospitalized with dysarthria and weakness of his right arm and leg. Three months previously, he had noticed numbness and weakness of his right shoulder, which spread to involve his left leg but which improved after 8 months. On admission, neurological examination revealed limb kinetic apraxia and constructive apraxia of the right hand, motor aphasia, dysarthria, and spastic quadriplegia. Sensory examination revealed hyperalgesia and dysesthesia in the right arm and left leg. Deep tendon reflexes were hyperactive in all four extremities. And he had bilateral Babinski signs. Laboratory examination revealed pH 7.38, PCO2 46.1 Torr, PO2 93.4 Torr, BE 1.7, and blood lactate, 9.0 mg/dl (normal 5-20 mg/dl). Cerebrospinal fluid lactate level was 20.0 mg/dl. pyruvate 1.34 mg/dl. and protein 83 mg/dl. Blood lactate and pyruvate values were markedly elevated after aerobic exercise. T2WI brain MRI showed scattered high signal lesions in the left precentral and postcentral gyrus, right paracentral lobes, both superior frontal gyri, and right superior temporal gyrus. Right biceps brachi biopsy showed almost complete cytochrome c oxidase (COX) deficiency. There were no ragged-red fibers. There was marked decrease of COX activity: 2.7 nmol/min/mg-mitochondrial protein (normal range: 33.0 +/- 16.1, n = 7) in the biopsied muscle. Open brain biopsy (after permission from the patient and his family) revealed gliosis and perivascular infiltration of lymphocytes and macrophages without vascular proliferation. There was no mitochondrial DNA mutations, deletion or duplication, including tRNA-Leu 3243, 8993, 3271, 9176, 3291, and tRNA-Lys 8344, 8356, and 8363. From these findings, a diagnosis of COX deficiency presenting as MELAS-like episodes was done. His mother also showed abnormality on aerobic exercise test, but she had no episode of stroke or neurological dysfunction. Six months later, his aphasia and apraxia of the right hand had resolved, and at discharge he was able to ambulate with a cane. Ten months later, he returned to his work. There has been no recurrence of neurologic symptoms over the next 3 years and 10 months. This patient appears to represent a rare case of adult onset COX deficiency presenting as MELAS-like episodes.

Adult↗
PubMed2004

[A case of myeloradiculitis as a complication of visceral larva migrans due to Ascaris suum].

A 35-year-old man noticed a tingling sensation and subsequent slight weakness in the distal part of the left lower extremities, which extended to the left thigh over the following 8 months, when he developed a urinary disturbance. He was admitted to a local hospital and diagnosed as having myelitis, because of the presence of a gadolinium (Gd)-enhanced lesion in the lumbar spinal cord corresponding to the tenth thoracic vertebra (Th10) level. The symptoms disappeared along with some residual sensory disturbance following intravenous administration of 1 g of methylprednisolone (IVMP) for 3 days. However, 4 months later, the patient gradually developed gait disturbance, dysuria, impotence, and fecal incontinence, and was admitted to our hospital in May 2001. A neurological examination revealed the presence of myeloradiculopathy causing a slight weakness in the left hamstring muscles, with positive Babinski and Lasegue signs on the same side. In addition, deep tendon reflexes were absent in the 4 extremities and vibration sense was moderately decreased in the lower extremities, though the results of electrophysiological tests of motor and sensory nerves were normal. A magnetic resonance imaging (MRI) study showed a Gd-enhanced lesion in the spinal cord at the Th8-9 vertebrae level, which was 1 to 2 vertebrae above the initially detected lesion. A lumbar puncture yielded cerebrospinal fluid (CSF) containing mononuclear cells at 7/mm3 that were comprised of an increasd number of CD4+CD25+ activated helper T cells; however, no myelin basic protein or oligoclonal IgG band was present. Serological examinations were negative for the presence of collagen/vascular disorders as well as viral infection due to CMV, EBV, HSV, VZV, and HTLV-1, however, positive for specific antibodies against Ascaris suum in both serum and the CSF, confirming the diagnosis of chronic myeloradiculitis due to visceral larva migrans. The patient was first treated with a single course of a daily 600-mg dose of oral albendazole for 4 weeks, which was resulted in restoration of muscle weakness, as well as disappearance of the Lasègue sign. However, in contrast to the clinical improvement, the CSF sample obtained immediately after completing the treatment showed a worsening trend, as the CSF cell count had increased with a manifestation of marked eosinophilia and CD4+CD25+ cells were also increased. Thereafter, 3-day IVMP treatment was performed twice in cojunction with 3 courses of oral albendazole therapy for the subsequent 4 months, which resulted in normalization of all laboratory measurements concerning the CSF along with a decrement trend in serum and CSF antibody titers specific to Ascaris suum. Our results suggest that neurological involvement due to visceral larva migrans can be efficaciously treated with not only helminthic drugs but also intravenous corticosteroids.

Adult↗
PubMed2004

[A case of transient left ventricular ballooning ("Takotsubo"-shaped cardiomyopathy) developed during plasmapheresis for treatment of myasthenic crisis].

An 83-year-old woman was admitted to our hospital complaining of respiratory distress. She developed blepharoptosis and dysphagia two months previously. On admission, blood pressure was 150/84 mmHg. There were bilateral blepharoptosis and bulbar palsy. Tendon reflexes were brisk, but pathological reflexes were absent. Repetitive stimulation test demonstrated decrement. Tensilon test was positive. Thus, a diagnosis of myasthenic crisis was made. Vital capacity was 0.71, which necessitated intubation and artificial ventilation. During the first course of immunoadsorption plasmapheresis, blood pressure fell to 80/50 mmHg. ECG showed deeply inverted T waves in leads V2 through V6. Echocardiographic examination demonstrated extensive akinesis around the apex. CK was slightly increased. A diagnosis of "Takotsubo"-shaped cardiomyopathy was made. To maintain blood pressure, dopamine was continuously injected. Left ventricular function returned to normal within a week. No thymoma was seen on the chest CT scan. She was treated with pyridostigmine and tacrolimus. Ten weeks after admission, she was weaned off the ventilator and she had no dysphagia anymore. Anti-acetylcholine receptor antibody titers had decreased. In Japan, approximately 17% of patients with myasthenia gravis are in the seventh decade or older. Approximately 10% of the patients of this age group suffer from severe generalized form. Careful ECG monitoring is necessary while treating elderly patients with myasthenic crisis, because they are at potential risk of developing "Takotsubo"-shaped cardiomyopathy.

Acute Disease↗
PubMed2004

[A case with late-onset MELAS with hallucination and delusion].

We report a 53-year-old male patient with late onset mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes(MELAS) with hallucination and delusion. The patient manifested various neurological symptoms including perceptive deafness, muscle weakness of limbs with loss of consciousness, sensory abnormalities in hands, feet and a face, abnormal sense of taste, tremor, palsy of upward eye movement and weak deep tendon reflexes prior to the psychotic episode. He was diagnosed as MELAS, because of high serum lactic acid and pyruvic acid, and the point mutation in the mitochondrial DNA 3243. SPECT imaging showed decreased perfusion in occipital cortex and thalamus. These SPECT changes improved after disappearing visual hallucination. Hallucination might be caused by delirium due to stroke-like episode. Dysfunction in the occipital cortex and thalamus might be involved with this perfusion change.

Delusions↗
PubMed2004

[High-dose intravenous immunoglobulin in the treatment of sensory ataxic neuropathy with Sjögren's syndrome: a case report].

We report herein a case of sensory ataxic neuropathy with Sjögren's syndrome (SS-SAN) who became dramatically improved in response to high-dose intravenous immunoglobulin treatment (IVIg). An 81-year-old man began to feel numbness in his hands and feet in August 2002. Because he became unsteady and could not do skillfull movement, he was admitted to our hospital in May 2003. On neurological examination, all tendon reflexes were absent. His vibratory and position senses were severely impaired to knees and elbows. Touch, temperature, and pinprick sensations were mildly disturbed in a glove-stocking distribution. Coordination was clumsy in all limbs because of sensory loss. He had gait ataxia with Romberg sign. Nerve conduction study revealed that sensory nerve action potentials were absent. He was diagnosed as having SS-SAN because Schirmer test, Saxon test and both SS-A and SS-B antibodies were positive. Thereafter, Mg, 400 mg/kg daily for 5 days, was administered. His sensory impairment began to improve 2 days after Mg. Subsequently, he could walk steadily without ataxia. It is considered that IVIg may be an effective treatment for SS-SAN.

Aged↗
PubMed2004

[Adult type tethered cord syndrome with chronic attackwise pain in the bilateral feet].

The authors report a case of chronic attackwise pain in the bilateral feet for five years due to tethered cord syndrome. Despite extensive examinations, this condition had been overlooked. The patient is a 21-year-old man. He had suffered attackwise pain resembling sticking a thumbtack in the soles of his feet, since he was 16 years old. The pain appeared several times a day and continued for 30 seconds to 30 minutes for 5 years. Physical examination revealed hammer toes and high-arched feet. The fingers and knee joints showed hyperextension. The neurological findings showed weakness of toe extension, hyporeflexia of deep tendon reflexes in the leg. Mild hypesthesia was seen in the bilateral soles. Myelography showed sacral dural ectasia. Magnetic resonance images showed dorsal displacement of the conus medullaris, the filum terminale and the cauda equina. A computed tomographic scan after myelography also showed a dorsally located thick filum terminale (the diameter is 2 mm). Surgery disclosed thick and tight filum terminale directly under the dura mater. Its flexibility was diminished. Abnormal lesions such as lipoma, spinal dysraphysm, diastematomyelia, myelomeningocele were not observed. After the untethering operation, the pain attacks decreased dramatically. The condition of the present case is adult onset tethered cord Group 2 described by Yamada. When unusual pain is manifested, we always have to keep this syndrome in mind.

Adult↗
PubMedDate not supplied

[A patient with mitochondrial encephalomyopathy presenting gynecomastia with elevation of serum estriol level].

We report a 23-year-old man with mitochondrial encephalomyopathy. At 21 years of age, he noted speech distubance. Since his dysarthria did not improve thereafter, he was admitted to our hospital. On admission, he showed mild gynecomastia. Neurological examination revealed mild decrease in performance IQ in WAIS-R, mild scanning speech, mild left hearing disturbance, mild to moderate muscle weakness in proximal four extremities, mild bilateral limb ataxia, and mild to moderate truncal ataxia. While, no brisk deep tendon reflex, pathological reflex, aberrant muscle tonus, sensory disturbance, retinopathy, myoclonus or autonomic disorder was found. Serum levels of lactate (23.2 mg/dl, normal<18.7) and pyruvate (1.23 mg/dl, normal<0.94) were elevated, and serum lactate levels were markedly elevated (118.1 mg/dl) after 15-minute exercise (15 Watts/minute). CSF levels of lactate (31.2 mg/dl, normal<12.5) and pyruvate (1.48 mg/dl, normal<0.75) were also elevated. Head MRI showed mild cerebral and cerebellar atrophy, but 1H-MRS showed no lactate peak. Moreover, muscle biopsy from left biceps muscle showed lots of ragged-red fibers, and he was thus diagnosed as having mitochondrial encephalomyopathy. However, nt3243 mutation of mitochondria DNA was not present. Next, we confirmed gynecomastia by mammography, and checked serum levels of estrogens. Mildly decreased estradiol (19.9 pg/ml, normal, 20-59), normal estrone (24.0 pg/ml, normal<30.0) and mildly increased estriol (6.03 pg/ml, normal<5.0) were found. While, the serum levels of cortisol, dehydroepiandrosterone-sulfate (DHEA-S), androstenedione, testosterone, luteinizing hormone (LH) and follicle stimulating hormone (FSH) were all within normal limits. Since the steroid hormone synthesis system and hypothalamus-pituitary system seem to be normal, 16alpha-hydroxylase that converts estradiol to estriol may be upregulated. While, aromatase (P-450arom) is well known to convert androgens to estrogens. In addition, 16alpha-hydroxylase and P-450arom convert DHEA-S to estriol. Since it is recently reported that P-450arom is considerably expressed in muscle tissues as well as fat tissues and that muscle tissue may be a major organ to produce estrogens in men and postmenopausal women, estriol production may be increased in the present patient's muscle. Although hypogonadism due to hypothalamus-pituitary disorders was sometimes reported, there have been no reports that suggest an increased estrogen production in skeletal muscles in mitochondrial encephalomyopathies. Recently, estrogen has been known to protect muscle fibers from oxidative damages due to exercise. Thus, it is of potential that estrogens increased locally in muscle tissues of the patients with mitochondrial encephalomyopathies protect muscle fibers from oxidative damage due to mitochondrial dysfunction.

Adult↗
PubMed2004

[A case of acute encephalopathy due to datura seed poisoning].

A 83 year-old-man presented with a stuporous state. He ingested datura seeds approximately 1.5 hour before the onset of the symptom. On arrival at our emergency clinic 2 hours and 40 minutes after the ingestion, he was semi-comatose with intermittent tonic convulsive seizures. The pupils were fully-dilated and unreactive, and salivation was decreased. He showed hypohidrosis and exaggerated deep tendon reflexes with positive Babinski's sign. His body temperature was 37.6 degrees C, and blood pressure was 167/99 mmHg. After gastrolavage and administration of charcoal and cathartics, his pupil became reactive to light, and he became alert gradually. His consciousness became clear 24 hours after seed ingestion and discharged with no residual neurological signs.

Acute Disease↗
PubMed2004

[Bulbovascular compression by megadolichobasilar artery manifested as neurogenic and refractory hypertension].

A 37-year-old man with juvenile and refractory hypertension was admitted to our hospital for progressive left hearing loss, vertigo, and dizziness. Neurological examination revealed left hearing loss and exaggerated deep tendon reflexes and vestibular dysfunction. MRI and cerebral angiography disclosed megadolichobasilar artery (MDBA). Moreover, modified MR cisternography at the medulla disclosed marked compression and deformity of the left rostral ventrolateral medulla (RVLM) by the dolichoectatic right vertebral artery. In the literatures, bulbovascular compression has been reported in 4 among 9 patients with MDBA (including the present patient), for whom MRI of the medulla was presented. All 4 patients suffered from hypertension, and at least 3 of them showed juvenile and refractory hypertension. Ipsilateral pyramidal tract disturbance (Opalski syndrome) was observed in 3 patients. Considering the recent concept that the cardiovascular center can be localized at the RVLM, juvenile and refractory hypertension, and possibly Opalski syndrome in the present patient can be attributed to bulbovascular compression by MDBA. In the patients with MDBA and hypertension or Opalski syndrome, MR cisternography of the medulla is warranted to evaluate compression by MDBA.

Adult↗