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Biomedical subjects

T E Bertorini

Publications and source records attributed to T E Bertorini.

At least 19 recordsLinked to original sources
PubMed2001

Progressive amyotrophy as a late complication of myelopathy.

A delayed syndrome of progressive weakness has been described in survivors of paralytic poliomyelitis - "Post-Polio Muscular Atrophy (PPMA)". One proposed etiology is a drop-out of motor neurons due to increased metabolic demands of an enlarged motor unit territory. We report a patient with slowly progressive lower extremity weakness 20 years after recovery from an episode of myelopathy involving the lower lumbar and sacral segments of the spinal cord. Delayed progressive amyotrophy may complicate any significant injury to anterior horn cells.

Adult↗
PubMed2001

HTLV-1 and its neurological complications.

BACKGROUND: Since its discovery in 1980, human T-cell lymphotropic virus type-1 (HTLV-1) has been associated with a number of neurological diseases. The distribution of HTLV-1-associated neurological disease is worldwide. In endemic areas, up to 30% of the population may be infected with HTLV-1; however, only a small percentage of infected persons develops neurological disease. REVIEW SUMMARY: In 1986, HTLV-1 infection was reported in patients of chronic progressive myelopathy of uncertain etiology, and the disease entity was called HTLV-1-associated myelopathy/tropical spastic paraparesis. Recently, HTLV-1 infection has been associated with polymyositis and uveitis. Interestingly, a single patient may display more than one syndrome. Although other neurological syndromes occur in HTLV-1-infected individuals, there is not enough epidemiologic data that show a strong association. Treatment of HTLV-1-associated neurological disease is challenging, and well-controlled studies are lacking. CONCLUSION: As neurologists and other scientists begin to understand the pathophysiology of HTLV-1 infection, improved therapies should be developed. Randomized trials with longer follow-up are required to understand the effect of treatment on disability and quality of life.

Journal Article↗
PubMed2000

Selective paraspinal muscle amyotrophy.

A 56-year-old female presented with mild low back pain. Examination revealed severe, selective atrophy of the thoracic and lumbar paraspinal muscles. Fibrillations were seen in the paraspinal muscles on EMG. Limb EMG was normal. Biopsy of the gluteus maximus was normal. Paraspinal muscle biopsy revealed neurogenic features. Atrophy of the thoracic and lumbar paraspinal muscles was noted on magnetic resonance imaging. This patient has selective amyotrophy of the thoracic and lumbar paraspinal muscles. This may be an addition to the spectrum of 'benign focal amyotrophy'. The differential diagnosis of paraspinal muscle weakness is discussed.

Biopsy↗
PubMed2000

Rapid growth of a basilar aneurysm.

We describe a patient who developed, over a 22-month period, a giant aneurysm of his basilar artery. A prior MRI of the brain done for nonspecific symptoms showed a normal brainstem and basilar artery. At presentation, he had a repeat MRI scan for a 4-month history of a partial right oculomotor nerve palsy and left hemiparesis. The MRI revealed a giant aneurysm of the top of the basilar artery. This was treated by angiographic placement of Guglielmi detachable coils (GDC) after surgical intervention was deemed unfeasible. This case illustrates the acquired nature of intracranial aneurysms. All inoperable intracranial aneurysms should be closely monitored and MRI and MR angiography may currently be the best noninvasive methods for this purpose. Intra-arterial GDC embolization of aneurysms is an alternative treatment when surgery is not possible.

Basilar Artery↗
PubMed2000

Long-term tracheostomy ventilation in neuromuscular diseases: patient acceptance and quality of life.

Domiciliary assisted ventilation has been used to prolong life in patients with neuromuscular diseases. Although earlier studies suggest that the majority of patients are satisfied with their lives, the physician's perception of a patient's poor quality of life on assisted ventilation is a major reason for discouraging assisted ventilation. In this study, the quality of life was assessed in 19 patients with neuromuscular diseases on domiciliary tracheal intermittent positive-pressure ventilation for a mean duration of 54 months. An attempt was made to compare the quality of life of Duchenne muscular dystrophy patients with that of amyotrophic lateral sclerosis patients. More than two-thirds of patients were satisfied with their lives. Eighty-four percent thought they had made the right choice. Patients with amyotrophic lateral sclerosis were somewhat more negative or ambiguous toward assisted ventilation and had lower life satisfaction scores as compared with Duchenne muscular dystrophy patients. Financial stresses were significant. Assisted ventilation should be offered as a viable option to patients with neuromuscular diseases. Larger studies may be useful in influencing insurance companies to make expenses associated with assisted ventilation reimbursable.

Adolescent↗
PubMed1999

Single-fiber electromyography of the laryngeal muscles.

Single-fiber electromyography (SFEMG) is useful in the evaluation of disorders of neuromuscular transmission and the assessment of motor unit morphology. Standard EMG techniques are used routinely in the evaluation of laryngeal dysfunction, but the feasibility of laryngeal SFEMG has not been established. We, therefore, performed laryngeal SFEMG in 10 normal individuals to demonstrate the feasibility of the technique and generate preliminary normative data. We also studied 2 patients with amyotrophic lateral sclerosis and 1 patient previously treated with botulinum toxin for comparative purposes.

Adult↗
PubMed1999

Focal myositis presenting with radial nerve palsy.

Focal myositis is a rare inflammatory pseudotumor of skeletal muscle which usually has a benign course. We report a 56-year-old woman with a painful mass in the left arm with a radial nerve palsy. Magnetic resonance imaging (MRI) of the left arm showed a mass in the triceps muscle that was suggestive of a soft-tissue sarcoma. Electromyography showed a severe radial neuropathy involving both motor and sensory axons. An open biopsy showed focal myositis. Treatment with corticosteroids resulted in complete disappearance of the mass clinically and by MRI, without recurrence for more than 2 years. Radial nerve function also recovered completely. As a treatable cause of focal neuropathy, focal myositis should be included in the differential diagnosis of a muscle mass.

Electromyography↗
PubMed1999

Bilateral ophthalmoplegia and exophthalmos complicating central hemodialysis catheter placement.

We describe a 58-year-old woman who presented with bilateral ophthalmoplegia, exophthalmos, and headache and was found to have retrograde internal jugular vein flow secondary to a high-grade obstruction of the ipsilateral brachiocephalic vein from a previous hemodialysis catheter placement. The patient had also a high-flow dialysis graft in the ipsilateral arm. The cranial and extracranial venous system congestion resolved, and the signs disappeared soon after a balloon angioplasty and stent placement at the level of the obstruction.

Angioplasty, Balloon↗
PubMed1998

Hypophosphatemia-induced neuropathy: clinical and electrophysiologic findings.

Severe hypophosphatemia has rarely been reported as a cause of acute paralysis. We present the clinical and electrophysiological findings on a patient who developed quadriparesis following several surgeries for complicated cholecystectomy. The paralysis was most likely the result of severe hypophosphatemia causing a neuropathy which improved readily after proper phosphate replacement. The possible pathogenic mechanisms of hypophosphatemic neuropathy are discussed.

Cholecystectomy↗
PubMed1998

Demyelinating neuropathy in a patient with multiple sclerosis and genotypical HMSN-1.

Patients with multiple sclerosis (MS) may develop a peripheral neuropathy, sometimes attributed to nutritional deficiency. Other patients present with a demyelinating neuropathy which is presumed to be the result of an autoimmune process that affects both the central and peripheral nervous systems. We report a case of concurring MS and demyelinating neuropathy, without a positive family history, in whom genetic testing proved the neuropathy to be hereditary and not autoimmune. Hereditary neuropathy should be a consideration in sporadic cases of peripheral neuropathy and MS.

Adult↗
PubMed1997

Remission of myasthenia gravis caused by proteinuria in nephrotic syndrome.

Myasthenia gravis is caused by antibodies against acetylcholine receptors and is treated with inhibition or elimination of antibody production. We report a 43-year-old myasthenic female who was symptomatic until she developed proteinuria from nephrotic syndrome, which caused a marked drop in acetylcholine receptor antibody titer with remission of myasthenia. Treatment of the nephrotic syndrome produced exacerbation of her myasthenia and a rise in antibody level. This patient's improvement is the result of antibody elimination during proteinuria in nephrotic syndrome.

Adult↗
PubMed1997

Neurologic and neuropsychiatric complications of Crohn's disease.

The literature contains sporadic accounts of neurologic complications occurring in patients with Crohn's Disease (CD). The pathogenesis of these is unknown and little has been reported about their incidence. Our review of the medical records of 253 patients with confirmed CD showed that neurologic and neuropsychiatric complications were evident in 84 of the patients, an incidence of 33.2%. In some the association could be casual, but in others the incidence of such complications was higher than that in the general population, suggesting a direct relationship with CD in 19.3%. Our study revealed a variety of neurologic and neuropsychologic events, such as seizure disorder, cerebrovascular accident, headache, peripheral neuropathy, myopathy, and major depression. We believe that an autoimmune phenomenon affecting the small vessels of the central and peripheral nerves may cause the most common neurologic complications of CD.

Adolescent↗
PubMed1997

Tongue involvement in a patient with granulomatous myositis.

A 64-year-old woman had difficulty swallowing and talking, weakness of the tongue, and progressive muscle weakness that was proven to be caused by a granulomatous myopathy. This case resembled a motor neuron disease, indicating that granulomatous myopathy should be considered in patients with similar presentations, since it is a treatable condition with a more benign prognosis.

Anti-Inflammatory Agents↗
PubMed1997

Encephalopathy due to capillary leak syndrome.

Systemic capillary leak syndrome (SCLS) is characterized by intermittent attacks of leakage of intravascular fluids into the extravascular space. Hypovolemia, hemoconcentration, weakness, edema, and visceral congestion are resulting manifestations of SCLS. Most patients with SCLS have clear mentation during attacks, and encephalopathy is not a known manifestation of the syndrome. We report a patient with acute idiopathic capillary leak syndrome manifested in an acute encephalopathy. The possibility of SCLS should be considered in patients who have an encephalopathy and hemoconcentration.

Brain Diseases↗