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PubMedDate not supplied

[A case of recurrent cerebral hemorrhage considered to be cerebral amyloid angiopathy by cerebrospinal fluid examination].

A 73-year-old man was admitted with gait disturbance and dysarthria. He showed right-side cerebellar ataxia. Computed tomography of brain showed left thalamic bleeding. Nine months later, he was admitted again because of seizure and consciousness disturbance. He had a history of diabetes mellitus and gout for five years, but no hypertension. On physical examination the lungs and heart were normal. On neurological examination, he showed stupor,pupils and eye position were normal. He showed right hemiparesis and urinary incontinence. The deep tendon reflexes were (+) at the upper limbs and (2+) at the right knee and ankle. Blood pressure was 162/88 mmHg and glucose was 275 mg/dl. Other laboratory data were normal. Brain CT showed hemorrhage of the left frontal lobe. The cystatin C level in cerebrospinal fluid was 68 ng/ml. Therefore we suspected cystatin C deposit amyloid angiopathy. In this case, thalamic hemorrhage was initially thought to be amyloid angiopathy. In cases of cerebral hemorrhage in the elderly without hypertension, we must be considered amyloid angiopathy.

Aged↗

PubMed1992

[A case of an aged patient suspected of craniopharyngioma with a chief symptom of eunuchoidism accompanied with panhypopituitarism].

A 78-year-old man with developmental disturbance of the genital organs and eunuchoidism was reported. He also had a high pitched voice, thickness of the lower lip and kyphosis of the thorax. He seemed to be fretful, but his intelligence was normal. Neurological tests revealed bilateral hemianopsia and decreased tendon reflexes. A plain skull radiograph clearly showed an egg shaped calcified mass extending upward from the sella turcica which resembled a ballooning shape. Brain CTs showed a high density round mass which expanded the sella turcica and raised the floor of the third ventricle. The inner part of the tumor showed irregular high density. T1-weighted MR imaging revealed an iso signal intensity, and T2 showed low signal intensity in the mass. These findings strongly supported the diagnosis of calcificated craniopharyngioma. Endocrinological study showed panhypopituitarism caused by the tumor compressing the pituitary gland and the hypothalamus. The main reasons why there were no apparent symptoms of hypopituitarism were because the receptors were up-regulated and secondarily because the thyroid and the adrenal cortical functions decreased while struggling to maintain balance with each other. There was also a possibility that these symptoms might have been masked by normal aging. Benign monoclonal hypergammopathy was also indicated, although we could not find a clear correlation between this finding and others.

Adrenocorticotropic Hormone↗

PubMed1993

[A case of primary systemic amyloidosis with skeletal muscle atrophy and congestive heart failure].

A 77-year-old male had been noticing progressive weakness of the legs for three years. By the age of 75 he had difficulty in climbing stairs. On admission, serum level of CPK was moderately high. There were weakness and atrophy of the proximal muscles. Deep tendon reflexes were depressed. Sensation was normal. The electromyogram and the biopsy of the femoral quadriceps muscles showed nonspecific changes. In 1989, he developed difficulty in walking and had congestive heart failure. On the second admission, moist rales were heard over the chest, and pitting edema was present in the lower extremities. The chest roentgenogram showed a cardiothoracic ratio of 63% and bilateral pleural effusion. The electrocardiogram showed atrial flutter with 2:1 conduction, QS in V1-3, rS in V4, and ST depression and T inversion in V5,6. The echocardiogram revealed a thick left ventricular wall and impaired left ventricular contraction (EF 22%). Macroglossia, hepatosplenomegaly and renal dysfunction were not noted. Congestive heart failure progressed and he suddenly died of ventricular tachycardia in December 1989. At autopsy, skeletal muscle fibers varied in size and showed fiber splitting. A cellular infiltration was observed in the stroma. Amyloid deposit was positively stained with Congo red. The heart weight was 570 g with marked left ventricular hypertrophy and moderate bilateral atrial dilatation. In both atria and ventricles, extensive amyloid deposition was found around myocardial fibers as well as in perivascular spaces. Amyloid was present also in the liver, the kidneys, the gastrointestinal tracts, and the other organs.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

PubMed1997

[Chronic neuropathy, a high level of protein in cerebrospinal fluid, and vitamin B1 and folate deficiency in a patient with normal-pressure hydrocephalus].

A 67-year-old woman presented with a 1-year history of gradual weight loss, reduced mental activity, muscle weakness, and urinary dysfunction. Neurological examination revealed mild lethargy, severe muscular atrophy, and diminished deep tendon reflexes in the extremities. The levels of vitamin B1 and folate in blood were low: 1.9 micrograms/dl (normal range 2.0-7.2) and 0.7 ng/ml (normal range 4.0-12.0). respectively. A lumbar puncture was done. The pressure of the cerebrospinal fluid was within normal limits, the level of protein was very high (467 mg/dl), and only a few lymphocytes were seen. A nerve-conduction study showed low amplitudes of action potentials and slow conduction velocities in both the motor and sensory nerves. Myelin irregularity, "onion bulb formation", and axonal atrophy were seen in a specimen obtained by sural nerve biopsy. A T2-weighted magnetic resonance image of the brain showed ventricular dilatation, high-intensity signals around the lateral ventricles, and a flow-void sign of the cerebral aqueduct. Radioisotope cisternography (111In-DTPA) disclosed ventricular reflux and slow clearance of the tracer from the ventricles. These findings indicated the presence of chronic inflammatory demyelinating polyneuropathy, nutritional polyneuropathy, vitamin B1 deficiency, folate deficiency, and normal pressure hydrocephalus. In this patient, the high level of protein in the cerebrospinal fluid may have caused the hydrocephalus.

Aged↗

PubMed1999

[An elderly case of juvenile muscular atrophy in the unilateral upper extremity with tremor in both hands].

A 75-year-old man had noticed muscle atrophy and weakness of his right hand and forearm at the age of 25. The symptoms slowly progressed and then stopped. Right hand tremor appeared at about age 40. There was no symptom in his left upper extremity, and his gait was normal. He now shows severe muscle atrophy in his right hand and forearm. There was distally dominant weakness of the right upper extremity and his hand grip was 0 kg on the right and 25 kg on the left. On admission there was no weakness in the bilateral lower extremities. He had postural tremor in both hands and fingers. The tendon reflexes were hypoactive in the upper extremities and normal in the lower extremities. Abnormalities in the superficial sensation were unremarkable, whereas vibration sensation in both the upper and lower extremities was mildly disturbed. Electromyography revealed chronic denervation, especially in the right upper extremity. The sensory nerve conduction study results and somatosensory evoked potentials in the upper extremities were normal. Cervical MRI demonstrated spondylotic changes, canal stenosis from the C5 to C7 levels, and compression of the spinal cord. His hand tremor was dominant on the right with a peak frequency of about 7 Hz. Tremor frequency and power were decreased by mechanical load. Hirayama's disease (juvenile muscular atrophy of unilateral upper extremity) was the most probable diagnosis, although aging might have produced various additional abnormalities. The tremor seen in this patient showed characteristics of enhanced physiological tremor.

Aged↗

PubMed2006

Intrathecal baclofen in the treatment of adult spasticity.

Medical management of adult spasticity, a condition of increased muscle tone and deep tendon reflexes, is often challenging and complex. Oral medications such as baclofen often have unacceptable supraspinal side effects at effective doses. Intrathecal baclofen delivered by an implanted catheter and pump system provides good relief of spasticity while overcoming these limitations. In this paper the authors survey the use of oral and intrathecal baclofen therapy, detail the surgical process, and explain the risks and benefits of the procedure.

Administration, Oral↗

PubMed1987

Thoracic myelopathy secondary to ossification of the spinal ligament.

The authors describe their experience with 26 cases of thoracic myelopathy secondary to hypertrophic ossification of the spinal ligament (posterior longitudinal ligament and/or ligamentum flavum). The clinical manifestations of this condition and results of its surgical treatment are described. The commonest symptoms were numbness or tingling in the legs and feet and gait disturbance. Most of the patients with involvement of the upper thoracic spine showed typical features of thoracic myelopathy: that is, sensory and motor deficits in both the trunk and lower extremities, sphincter disturbance, and exaggerated tendon reflexes. Several patients with involvement of the thoracolumbar junction presented with atypical symptoms of thoracic myelopathy and were sometimes misdiagnosed and treated inappropriately. Surgical treatment, particularly laminectomy, was not always successful. Inconsistencies in the surgical outcome were caused by either operative complications or reversal of the initial improvement during the follow-up period. The results of anterior surgery for the condition were more favorable; however, use of this procedure was rarely indicated.

Adult↗

PubMed1988

Changes in radicular function following low-back surgery.

Results of neurological assessment 1 year following surgical treatment of herniated lumbar intervertebral discs and lumbar stenosis are reported in 443 patients. The data were collected using separate series of questionnaires to be completed by the patient and the surgeon. Preoperatively, motor loss was reported by 12% of patients, while surgeons found motor weakness in 28%. Postoperative motor loss was still present in 24% and 25% of these cases, respectively; the operation had caused or aggravated motor loss in 5% and 3% of cases, respectively. Sensory phenomena were reported by 53% of the patients, while surgeons found sensory loss in 45%. Sensation was reported as abnormal 1 year after surgery both by patients and by surgeons in one-third of these cases. Sensory loss, considered by the patient to be caused or aggravated by operation, occurred in 15% of cases and in 12% of cases the surgeons agreed. Preoperatively, unilaterally diminished knee and Achilles tendon reflexes were found in 9% and 42%, respectively; at 1 year after surgery, these had recovered in 65% and 57% of cases, respectively. Surgery caused or aggravated unilaterally diminished knee or ankle jerks in 3% and 10% of cases, respectively.

Humans↗

PubMed1998

Acute quadriplegia with delayed onset and rapid recovery. Case report.

The authors describe a patient with severe head injury and sepsis who became acutely quadriplegic 3 days postinjury because of a critical illness polyneuropathy (CIP) and critical illness myopathy (CIM), which resolved rapidly after treatment of the underlying infection. In only 3 days the patient developed septic shock together with flaccid quadriplegia and absent deep tendon reflexes with no clinical or radiological evidence of central nervous system deterioration. Neurophysiological studies showed an acute axonal sensorimotor polyneuropathy, whereas the clinical course strongly suggested a concurrent myopathy. A severe Staphylococcus epidermidis infection accompanied by bacteremia was treated and the patient recovered fully within a few days. Although the case described here is unique because of its very early onset and rapid resolution, CIP and CIM are frequent complications of sepsis and multiple organ failure. The authors suggest that severely head injured patients with sepsis should be evaluated for CIP and CIM when presenting with unexplained muscle weakness or paralysis.

Acute Disease↗

PubMed1999

Lobular capillary hemangioma of the cauda equina. Case report.

This 56-year-old woman presented with a 1-year history of low-back pain, sciatica, and paresthesias in the right S-1 dermatome. On examination the patient was shown to have a right-sided Lasegue's sign, normal strength, hypalgesia in the right S-1 dermatome, and a slight diminution of the right Achilles tendon reflex. Magnetic resonance imaging revealed a 2-cm intradural enhancing lesion at the level of the L-4 vertebra. Laminectomy of L3-L5 vertebrae was performed, and intradural exploration disclosed a blueberry-appearing tumor that was surrounded by an intense arachnoiditis and attached to the right S-1 nerve root. A cystic collection of cerebrospinal fluid was seen caudal to the tumor. Complete removal required transection of the adherent nerve root fascicles. Histological analyses indicate that the lesion was a lobular capillary hemangioma, which, to the authors' knowledge, appears to be one of the first recorded examples of such a case.

Cauda Equina↗

PubMed2005

Usefulness of neurological examination for diagnosis of the affected level in patients with cervical compressive myelopathy: prospective comparative study with radiological evaluation.

OBJECT: Although neurological examination is the key step to reaching a correct diagnosis of cervical compressive myelopathy (CCM), the accuracy of diagnosis of the affected spinal level for CCM has not yet been tested. METHODS: The authors conducted a prospective study to elucidate how accurately the affected intervertebral level can be determined and decompressed based on neurological examination. Fifty patients who underwent successful decompressive surgery for cervical myelopathy caused by single-level disc herniation or spondylosis were included in this study (38 men and 12 women, mean age 60 years). Three board-certified spine surgeons participated in establishing the neurological diagnoses. One of the three surgeons made a diagnosis of CCM, and the other two conducted the neurological examination including deep tendon reflex, pinprick response, muscle weakness, and numbness in the hand only, knowing that the patient had CCM, and established the neurological-level diagnosis. A single intervertebral level responsible for patient's symptoms was determined concordantly based on magnetic resonance imaging and myelography findings by two spine surgeons, and this served as the standard. Agreement between neurological and neuroimaging/radiological level diagnoses was determined. The rate of agreement between neurological and neuroimaging diagnosis was 66%. Among the neurological tests, patient-perceived location of numbness in the hands was the most useful for establishing the affected level. For the other three tests the agreement rate was lower than 50% and thus each individual test may not be reliable for diagnosing the affected level. CONCLUSIONS: The results of this study suggested that neurological examination in patients with CCM is moderately accurate and reliable for determining the neurological level of disease.

Adult↗

PubMedDate not supplied

Adie's syndrome as a cause of amblyopia.

Adie's syndrome comprises a tonic pupil, which may be associated with impairment of accommodation, in the presence of diminished or absent deep tendon reflexes. We report a case of a 4-year-old boy with Adie's syndrome in which latent hypermetropia was made manifest by accommodative paresis and resulted in reversible amblyopia.

Accommodation, Ocular↗

PubMed2006

[Idiopathic Lambert-Eaton myasthenic syndrome: report of one case].

Lambert-Eaton myasthenic syndrome (LEMS) is an autoinmune idiopathic or paraneoplastic syndrome producing antibodies agaisnt presynaptic voltage calcium channels. The clinical features of patients with LEMS are muscle weakness and autonomic dysfunction. We report a 40 years old man with a four years history of proximal weakness, absent tendon reflexes and dry mouth. The diagnosis was confirmed by characteristic electromyographic findings, showing a low-amplitude muscle response that increased dramatically after activation. Circulating antibodies to voltage-gated calcium channel were present. The search for malignant tumors was negative. The patient was treated with prednisone and azathioprine and after four months, he was able to walk and signs of autonomic dysfunction started to subside.

Adult↗

PubMedDate not supplied

Evidence for neuronal localisation of enteroviral sequences in motor neurone disease/amyotrophic lateral sclerosis by in situ hybridization.

Sequences resembling those of human enterovirus type B sequences have been associated with motor neurone disease/amyotrophic lateral sclerosis. In a previous study we detected enteroviral sequences in spinal cord/brain stem from cases of motor neurone disease/amyotrophic lateral sclerosis, but not controls. Adjacent tissue sections to two of those strongly positive for these sequences by reverse-transcriptase polymerase chain reaction were analyzed by in situ hybridization with digoxigenin-labelled virus-specific antisense riboprobes. In one case, a female aged 83 showing 12 month rapid progressive disease, signal was specifically localized to cells identifiable as motor neurones of the anterior horn. In another case, a male aged 63 with a 60-month history of progressive muscle weakness, dysarthia, dyspnoea and increased tendon reflexes, signal was located to neurones in the gracile/cuneate nuclei of the brain stem tissue block that had been analyzed. This case showed loss of neurones in the anterior horn of the spinal cord by histopathologic examination which would account for clinical signs of motor neurone disease/amyotrophic lateral sclerosis. Dysfunction of the gracile/cuneate nuclei might have been masked by the paralytic disease. These structures are adjacent to the hypoglossal nuclei, and suggest either localised dissemination from hypoglossal nuclei or a possible route of dissemination of infection through the brainstem to the hypoglossal nuclei. These findings provide further evidence for the possible involvement of enteroviruses in motor neurone disease/amyotrophic lateral sclerosis.

5' Untranslated Regions↗

PubMed2000

Craniovertebral junction anomalies seen at Kenyatta National Hospital, Nairobi.

OBJECTIVE: To study the frequency, mode of presentation and outcome following surgery of anomalies of the craniovertebral junction at the Kenyatta National Hospital. DESIGN: A retrospective study. SETTING: Kenyatta National Hospital, Nairobi. SUBJECTS: Thirty eight patients with craniovertebral junction anomaly who were treated at the Kenyatta National Hospital. RESULTS: Thirty eight patients were diagnosed to have craniovertebral junction anomaly at the Kenyatta National Hospital between January 1988 and December 1994. Thirty of those patients underwent surgery. The ages ranged between 10 years and 49 years with a peak in the third decade. The male to female ratio was 2:1. The common mode of presentation was weakness of the extremities (90%) followed by sensory disturbances such as numbness of the extremities (30%), headache (30%) and neck pain (20%). The common clinical findings were ataxia, muscle wasting, sensory loss and brisk deep tendon reflexes. Most of the patients had computerised tomography (CT) scanning (80%) done. Other radiological investigations which were done were plain x-rays of the skull and cervical spine (50%), myelographic studies (36%) and tomographic studies (12%). Forty percent of the patients improved following surgery while further neurological deterioration was prevented in 48%. There was an immediate post-operative mortality of 8%. CONCLUSION: Craniovertebral junction anomaly is an important clinicopathological, condition common amongst certain ethnic groups found in high altitude areas of the Kenya. Surgery may cause clinical improvement in 40% of the patients, mainly those who have been symptomatic for less than three years.

Adolescent↗

PubMed2001

Hydroquinone neuropathy following use of skin bleaching creams: case report.

A 30-year old black woman presented with gradual onset of weakness of the legs associated with burning sensation in the feet for two months. She had been using two hydroquinone based skin bleaching creams (MGC by M. G. C. International, MEKAKO by Anglo Fabrics BOLTON Ltd) for about four years. Her BP was 80/40 mm Hg supine with un-recordable diastolic pressure on standing. She had decreased power (Grade 3/5), loss of deep tendon reflexes and impairment of deep sensation in the lower limbs. A complete blood count, urinalysis, serum electrolytes, serum creatinine and uric acid were all normal. Oral GTT, VDRL and brucella tests were negative. Chest and abdominal radiographs did not show any abnormalities. A diagnosis of peripheral neuropathy with autonomic neuropathy possibly due to hydroquinone toxicity was made and she was advised to stop using hydroquinone based skin bleaching creams. Four months later she was asymptomatic, her BP was 120/80 mmHg supine and standing, and neurological examination was normal. The case raises the question of whether hydroquinone based skin bleaching creams could be a cause of peripheral neuropathy and underscores the need for research on hydroquinone based skin bleaching creams and neuropathy particularly in black women involved in the sale and/or use of skin bleaching creams.

Adult↗

PubMed2005

Peripheral nerve function and symptom distress during biotherapy for malignant melanoma.

Studies of biotherapy-induced physiological changes are few, and systematic monitoring for neurotoxic effects are lacking. The purpose of this exploratory pilot study was to determine the change in peripheral nerve function and symptom distress during treatment with biotherapy for malignant melanoma. A convenience sample of 11 participants with malignant melanoma receiving interferon-alpha had measures of peripheral nerve function measured at baseline, four and 12 weeks of treatment. Data were analyzed using plots and regression slopes to determine change over time in sensation, gait/balance, vision, hearing, vibratory sense, muscle strength, deep tendon reflexes, blood pressure, and symptom distress. Declines in hearing, sensation, vibration, and muscle strength were found. Changes in visual acuity, and orthostatic blood pressure were noted, while gait/balance remained stable. Additionally, neuropathy symptoms were associated with symptom distress. The characterization of such changes can increase our understanding of the nature of the physiological effects associated with high-dose biotherapy treatment and aid clinicians to better prepare patients for anticipated changes in function and subsequent lifestyle adjustments. These findings can be used to provide information in a larger study of this phenomenon regarding important outcomes and measurement time-points of therapy-induced neuropathy and decreasing symptom distress in patients receiving cancer treatment with biologic agents.

Adult↗

PubMed1992

Neurological examination of patients suffering from leprosy: is it worthwhile?

We examined 28 male leprosy patients to discover if a more extensive neurological investigation than usual would be worthwhile in diagnosis and/or management. Our findings were fully compatible with what might be expected from a mononeuritis multiplex, either due to leprosy or other causes. The following observations are noteworthy. Changes of position sense and a decrease of some tendon reflexes were present in a minority of the patients. In soles of the feet, considered to be an- or hypaesthetic, some residual pain sensation could occasionally be detected. Functional testing of at least one muscle group (m. triceps surae) appeared to be more reliable than manual testing according to MRC criteria. We concluded that an extensive neurological examination is probably not required for diagnosis. It does provide, however, more accurate information on the extent of damage to the peripheral nervous system, which may be important for management and for assessment of treatment effects. The use of a myometer is advocated.

Adult↗