Speciality
Spotlight

 




 


Neurology


 

 





Neuromuscular
Disease

       

  • SC Apfel, JA Kessler, BT Adornato, et al (Albert Einstein College of Medicine, Bronx, NY; Stanford Univ, Palo Atlo, CA; Health Partners, Minneapolis; et al)


    Recombinant Human Nerve Growth Factor in the Treatment of Diabetic Polyneuropathy.


    Neurology 51: 695-702, 1998.


      


    250 patients with symptomatic diabetic polyneuropathy were assigned randomly to placebo or to two doses of recombinant Human Nerve Growth Factor (rhNGF) for 6 months. Symptoms and signs of polyneuropathy were assessed before and after treatment.

      


    Preliminary findings suggest that rhNGF is an effective and safe treatment for symptomatic diabetic
    polyneuropathy.

       

  • M Stangel, H-P Hartung, P Marx, et al (Universitatsklinikum Benjamin Franklin, Hindenburgdamm, Berlin; Univ of Wurzburg, Germany; Karl-Franzeins Universitat, Gruz, Austria)


    Intravenous Immunoglobulin Treatment of Neurological Autoimmune Diseases


    J Neurol Sci 153: 203-214, 1998

       


    Commercially available intravenous immunoglobulin (IVIg) preparations are created from a pool of more than 5000 healthy donors, which guarantees diversity of immunoglobulins. Only select donors are accepted and are routinely screened for antibodies to HIV, HCV, syphilis and hepatitis-B antigen.

       


    Use of IVIg is safer (than plasma exchange) in patients with hypotension or autonomic instability. Contraindications for IVIg use are selective IgA deficiency, anaphylactic episode after previous IVIg infusion, congestive heart failure and renal insufficiency.

        


    Neurologic autoimmune diseases for which there is evidence to support treatment with IVIg include Guillain-Barre syndrome chronic inflammatory demyelinating neuropathy, multifocal motor neuropathy and polyneuropathy. Favourable results have been reported in cases of myasthenia gravis and
    myositis.

       

  • SV Rajkumar, MA Gertz, RA Kyle (Mayo Clinic and Mayo Found, Rochester, Minn)


    Prognosis of Patients with Primary Systemic Amyloidosis Who Present With Dominant Neuropathy.



    Am J Med 104: 232-237, 1998.


        


    Peripheral nerve involvement, a well-described characteristic of primary systemic amyloidosis, is the initial manifestation of disease in some patients.

       


    Primary systemic amyloidosis is associated with the deposition of fragments of immunoglobulin light chains in a variety of tissues.

        


    The outcome of disease was assessed in 26 patients with sural nerve biopsy-proven amyloid neuropathy and monoclonal proteins in the serum or urine. These 26 patients met all inclusion criteria from a group of 1282 patients diagnosed (with dominant neuropathy) between 01-01-1978 and 31-12-1994.

       


    The commonest neurologic symptoms were paresthesias (81% patients), muscle weakness (65% patients) and numbness (58%).

        


    Diagnosis of primary systemic amyloidosis is often delayed. The median time to diagnosis is longer than survival after diagnosis. About half to two-thirds of patients have symptoms of autonomic involvement. Patients become immobile or bedridden due to progress of neuropathy. Therapy does not improve neuropathy.

       

  • M
    Lofberg, H Jankala, A Paetau, et al (Helsinki Univ)


    Metabolic Causes of Recurrent Rhabdomyolysis



    Acta Neurol Scand 98: 268-275, 1998.


       


    Rhabdomyolysis is characterized by acute destruction of skeletal muscle, which results in myoglobinuria and risk of renal damage.

       


    The majority of cases seem to be caused by mechanical or vascular damage. Some result from an underlying metabolic defect, often in patients with recurrent rhabdomyolysis with incidents preceded by exercise or infection.

       


    The biochemical causes of recurrent rhabdomyolysis were assessed.

        


    Nearly one fourth of the patients with recurrent rhabdomyolysis were found to have enzyme defects. Eighteen percent of these patients had other muscle diseases, muscular dystrophies or
    myopathies.

       

  • D Berg, W Mullges, M Koltzenburg, et al (Bayerische Julius-Maximilians-Universitat, Wurzburg, Germany)


    Man-in-the-Barrel Syndrome Caused by Cervical Spinal Cord Infarction.



    Acta Neurol Scand 97: 417-419, 1998.


        


    Man-in-the-barrel (MIB) syndrome, or acute brachial diplegia with normal findings of the lower extremities is usually caused by a lesion located bilaterally at the border zone between the anterior and middle cerebral artery territories.

         


    This case report stresses that for patients with brachial diplegia with normal supracervical findings, an atypical cervical cord INFARCTION should be considered even if the patient is comatose and has normal inferior extremities.

      

  • SC
    Apfel, JA Kessler, BT Adornato, et al (Albert
    Einstein College of Medicine, Bronx, NY; Stanford
    Univ, Palo Atlo, CA; Health Partners, Minneapolis;
    et al)

    Recombinant
    Human Nerve Growth Factor in the Treatment of
    Diabetic Polyneuropathy.

    Neurology
    51: 695-702, 1998.

     

    250
    patients with symptomatic diabetic polyneuropathy
    were assigned randomly to placebo or to two doses of
    recombinant Human Nerve Growth Factor (rhNGF) for 6
    months. Symptoms and signs of polyneuropathy were assessed before and
    after treatment.

     

    Preliminary
    findings suggest that rhNGF is an effective and safe
    treatment for symptomatic diabetic polyneuropathy.

        

  • M
    Stangel, H-P Hartung, P Marx, et al (Universitatsklinikum
    Benjamin Franklin, Hindenburgdamm, Berlin; Univ of
    Wurzburg, Germany; Karl-Franzeins Universitat, Gruz,
    Austria)

    Intravenous
    Immunoglobulin Treatment of Neurological Autoimmune
    Diseases

    J Neurol Sci 153: 203-214, 1998

       

    Commercially
    available intravenous immunoglobulin (IVIg)
    preparations are created from a pool of more than
    5000 healthy donors, which guarantees diversity of
    immunoglobulins. Only
    select donors are accepted and are routinely
    screened for antibodies to HIV, HCV, syphilis and
    hepatitis-B antigen.

     

    Use
    of IVIg is safer (than plasma exchange) in patients
    with hypotension or autonomic instability. Contraindications for IVIg use are selective IgA
    deficiency, anaphylactic episode after previous IVIg
    infusion, congestive heart failure and renal
    insufficiency.

         

    Neurologic autoimmune diseases for which there is
    evidence to support treatment with IVIg include
    Guillain-Barre syndrome chronic inflammatory
    demyelinating neuropathy,
    multifocal motor neuropathy and polyneuropathy. Favourable results have been reported in cases of myasthenia gravis
    and myositis.


         

  • SV
    Rajkumar, MA Gertz, RA Kyle (Mayo Clinic and Mayo
    Found, Rochester, Minn)

    Prognosis
    of Patients with Primary Systemic Amyloidosis Who
    Present With Dominant Neuropathy.

    Am J Med 104: 232-237, 1998.

      

    Peripheral nerve involvement, a well-described
    characteristic of primary systemic amyloidosis, is
    the initial manifestation of disease in some
    patients.

     

    Primary
    systemic amyloidosis is associated with the
    deposition of fragments of immunoglobulin light
    chains in a variety of tissues.

     

    The
    outcome of disease was assessed in 26 patients with
    sural nerve biopsy-proven amyloid neuropathy and
    monoclonal proteins in the serum or urine.
    These 26 patients met all inclusion criteria
    from a group of 1282 patients diagnosed (with
    dominant neuropathy) between 01-01-1978 and
    31-12-1994.

     

    The
    commonest neurologic symptoms were paresthesias (81%
    patients), muscle weakness (65% patients) and
    numbness (58%).

     

    Diagnosis
    of primary systemic amyloidosis is often delayed.
    The median time to diagnosis is longer than
    survival after diagnosis.  About half to two-thirds of patients have symptoms of
    autonomic involvement.
    Patients become immobile or bedridden due to
    progress of neuropathy.
    Therapy does not improve neuropathy.

       

  • M
    Lofberg, H Jankala, A Paetau, et al (Helsinki Univ)

    Metabolic
    Causes of Recurrent Rhabdomyolysis

    Acta Neurol Scand 98: 268-275, 1998.

       

    Rhabdomyolysis is characterized by acute
    destruction of skeletal muscle, which results in
    myoglobinuria and risk of renal damage.

        

    The
    majority of cases seem to be caused by mechanical or
    vascular damage. Some
    result from an underlying metabolic defect, often in
    patients with recurrent rhabdomyolysis with
    incidents preceded by exercise or infection.

       

    The
    biochemical causes of recurrent rhabdomyolysis were
    assessed.

      

    Nearly
    one fourth of the patients with recurrent
    rhabdomyolysis were found to have enzyme defects. Eighteen percent of these patients had other muscle diseases,
    muscular dystrophies or myopathies.

         

  • D
    Berg, W Mullges, M Koltzenburg, et al (Bayerische
    Julius-Maximilians-Universitat, Wurzburg, Germany)

    Man-in-the-Barrel
    Syndrome Caused by Cervical Spinal Cord Infarction.

    Acta
    Neurol Scand 97: 417-419, 1998.

      

    Man-in-the-barrel (MIB) syndrome, or acute brachial
    diplegia with normal findings of the lower
    extremities is usually caused by a lesion located
    bilaterally at the border zone between the anterior
    and middle cerebral artery territories.

     

    This
    case report stresses that for patients with brachial
    diplegia with normal supracervical findings, an
    atypical cervical cord INFARCTIOn
    should be considered even if the patient is comatose
    and has normal inferior extremities.

      

  • MG Edward, P Sampath, JA Williams et al (Univ of North Carolina, Chapel Hill; Johns Hopkins Med Institutions, Baltimore,
    Md).

      Local Delivery of Chemotherapy Prolongs Survival in Experimental Brain Metastases from Breast Carcinoma.

      Neurosurgery 43: 1185-1193, 1998.

        

    Local delivery of carmustine with biodegradable polymers can significantly prolong survival in a murine model of intracranial metastatic breast cancer. The incidence of local recurrence of breast cancer metastases with minimal morbidity may be decreased with surgical resection and placement of carmustine polymers into the resection cavity. 

        

 



 

 

Speciality Spotlight

 

 
Neurology
 

 

Neuromuscular Disease
       

  • SC Apfel, JA Kessler, BT Adornato, et al (Albert Einstein College of Medicine, Bronx, NY; Stanford Univ, Palo Atlo, CA; Health Partners, Minneapolis; et al)
    Recombinant Human Nerve Growth Factor in the Treatment of Diabetic Polyneuropathy.
    Neurology 51: 695-702, 1998.
      
    250 patients with symptomatic diabetic polyneuropathy were assigned randomly to placebo or to two doses of recombinant Human Nerve Growth Factor (rhNGF) for 6 months. Symptoms and signs of polyneuropathy were assessed before and after treatment.
      
    Preliminary findings suggest that rhNGF is an effective and safe treatment for symptomatic diabetic polyneuropathy.
       

  • M Stangel, H-P Hartung, P Marx, et al (Universitatsklinikum Benjamin Franklin, Hindenburgdamm, Berlin; Univ of Wurzburg, Germany; Karl-Franzeins Universitat, Gruz, Austria)
    Intravenous Immunoglobulin Treatment of Neurological Autoimmune Diseases
    J Neurol Sci 153: 203-214, 1998
       
    Commercially available intravenous immunoglobulin (IVIg) preparations are created from a pool of more than 5000 healthy donors, which guarantees diversity of immunoglobulins. Only select donors are accepted and are routinely screened for antibodies to HIV, HCV, syphilis and hepatitis-B antigen.
       
    Use of IVIg is safer (than plasma exchange) in patients with hypotension or autonomic instability. Contraindications for IVIg use are selective IgA deficiency, anaphylactic episode after previous IVIg infusion, congestive heart failure and renal insufficiency.
        
    Neurologic autoimmune diseases for which there is evidence to support treatment with IVIg include Guillain-Barre syndrome chronic inflammatory demyelinating neuropathy, multifocal motor neuropathy and polyneuropathy. Favourable results have been reported in cases of myasthenia gravis and myositis.
       

  • SV Rajkumar, MA Gertz, RA Kyle (Mayo Clinic and Mayo Found, Rochester, Minn)
    Prognosis of Patients with Primary Systemic Amyloidosis Who Present With Dominant Neuropathy.
    Am J Med 104: 232-237, 1998.
        
    Peripheral nerve involvement, a well-described characteristic of primary systemic amyloidosis, is the initial manifestation of disease in some patients.
       
    Primary systemic amyloidosis is associated with the deposition of fragments of immunoglobulin light chains in a variety of tissues.
        
    The outcome of disease was assessed in 26 patients with sural nerve biopsy-proven amyloid neuropathy and monoclonal proteins in the serum or urine. These 26 patients met all inclusion criteria from a group of 1282 patients diagnosed (with dominant neuropathy) between 01-01-1978 and 31-12-1994.
       
    The commonest neurologic symptoms were paresthesias (81% patients), muscle weakness (65% patients) and numbness (58%).
        
    Diagnosis of primary systemic amyloidosis is often delayed. The median time to diagnosis is longer than survival after diagnosis. About half to two-thirds of patients have symptoms of autonomic involvement. Patients become immobile or bedridden due to progress of neuropathy. Therapy does not improve neuropathy.
       

  • M Lofberg, H Jankala, A Paetau, et al (Helsinki Univ)
    Metabolic Causes of Recurrent Rhabdomyolysis
    Acta Neurol Scand 98: 268-275, 1998.
       
    Rhabdomyolysis is characterized by acute destruction of skeletal muscle, which results in myoglobinuria and risk of renal damage.
       
    The majority of cases seem to be caused by mechanical or vascular damage. Some result from an underlying metabolic defect, often in patients with recurrent rhabdomyolysis with incidents preceded by exercise or infection.
       
    The biochemical causes of recurrent rhabdomyolysis were assessed.
        
    Nearly one fourth of the patients with recurrent rhabdomyolysis were found to have enzyme defects. Eighteen percent of these patients had other muscle diseases, muscular dystrophies or myopathies.
       

  • D Berg, W Mullges, M Koltzenburg, et al (Bayerische Julius-Maximilians-Universitat, Wurzburg, Germany)
    Man-in-the-Barrel Syndrome Caused by Cervical Spinal Cord Infarction.
    Acta Neurol Scand 97: 417-419, 1998.
        
    Man-in-the-barrel (MIB) syndrome, or acute brachial diplegia with normal findings of the lower extremities is usually caused by a lesion located bilaterally at the border zone between the anterior and middle cerebral artery territories.
         
    This case report stresses that for patients with brachial diplegia with normal supracervical findings, an atypical cervical cord INFARCTION should be considered even if the patient is comatose and has normal inferior extremities.
      

  • SC Apfel, JA Kessler, BT Adornato, et al (Albert Einstein College of Medicine, Bronx, NY; Stanford Univ, Palo Atlo, CA; Health Partners, Minneapolis; et al)
    Recombinant Human Nerve Growth Factor in the Treatment of Diabetic Polyneuropathy.
    Neurology 51: 695-702, 1998.
     
    250 patients with symptomatic diabetic polyneuropathy were assigned randomly to placebo or to two doses of recombinant Human Nerve Growth Factor (rhNGF) for 6 months. Symptoms and signs of polyneuropathy were assessed before and after treatment.
     
    Preliminary findings suggest that rhNGF is an effective and safe treatment for symptomatic diabetic polyneuropathy.
        

  • M Stangel, H-P Hartung, P Marx, et al (Universitatsklinikum Benjamin Franklin, Hindenburgdamm, Berlin; Univ of Wurzburg, Germany; Karl-Franzeins Universitat, Gruz, Austria)
    Intravenous Immunoglobulin Treatment of Neurological Autoimmune Diseases
    J Neurol Sci 153: 203-214, 1998
       
    Commercially available intravenous immunoglobulin (IVIg) preparations are created from a pool of more than 5000 healthy donors, which guarantees diversity of immunoglobulins. Only select donors are accepted and are routinely screened for antibodies to HIV, HCV, syphilis and hepatitis-B antigen.
     
    Use of IVIg is safer (than plasma exchange) in patients with hypotension or autonomic instability. Contraindications for IVIg use are selective IgA deficiency, anaphylactic episode after previous IVIg infusion, congestive heart failure and renal insufficiency.
         
    Neurologic autoimmune diseases for which there is evidence to support treatment with IVIg include Guillain-Barre syndrome chronic inflammatory demyelinating neuropathy, multifocal motor neuropathy and polyneuropathy. Favourable results have been reported in cases of myasthenia gravis and myositis.
         

  • SV Rajkumar, MA Gertz, RA Kyle (Mayo Clinic and Mayo Found, Rochester, Minn)
    Prognosis of Patients with Primary Systemic Amyloidosis Who Present With Dominant Neuropathy.
    Am J Med 104: 232-237, 1998.
      
    Peripheral nerve involvement, a well-described characteristic of primary systemic amyloidosis, is the initial manifestation of disease in some patients.
     
    Primary systemic amyloidosis is associated with the deposition of fragments of immunoglobulin light chains in a variety of tissues.
     
    The outcome of disease was assessed in 26 patients with sural nerve biopsy-proven amyloid neuropathy and monoclonal proteins in the serum or urine. These 26 patients met all inclusion criteria from a group of 1282 patients diagnosed (with dominant neuropathy) between 01-01-1978 and 31-12-1994.
     
    The commonest neurologic symptoms were paresthesias (81% patients), muscle weakness (65% patients) and numbness (58%).
     
    Diagnosis of primary systemic amyloidosis is often delayed. The median time to diagnosis is longer than survival after diagnosis.  About half to two-thirds of patients have symptoms of autonomic involvement. Patients become immobile or bedridden due to progress of neuropathy. Therapy does not improve neuropathy.
       

  • M Lofberg, H Jankala, A Paetau, et al (Helsinki Univ)
    Metabolic Causes of Recurrent Rhabdomyolysis
    Acta Neurol Scand 98: 268-275, 1998.
       
    Rhabdomyolysis is characterized by acute destruction of skeletal muscle, which results in myoglobinuria and risk of renal damage.
        
    The majority of cases seem to be caused by mechanical or vascular damage. Some result from an underlying metabolic defect, often in patients with recurrent rhabdomyolysis with incidents preceded by exercise or infection.
       
    The biochemical causes of recurrent rhabdomyolysis were assessed.
      
    Nearly one fourth of the patients with recurrent rhabdomyolysis were found to have enzyme defects. Eighteen percent of these patients had other muscle diseases, muscular dystrophies or myopathies.
         

  • D Berg, W Mullges, M Koltzenburg, et al (Bayerische Julius-Maximilians-Universitat, Wurzburg, Germany)
    Man-in-the-Barrel Syndrome Caused by Cervical Spinal Cord Infarction.
    Acta Neurol Scand 97: 417-419, 1998.
      
    Man-in-the-barrel (MIB) syndrome, or acute brachial diplegia with normal findings of the lower extremities is usually caused by a lesion located bilaterally at the border zone between the anterior and middle cerebral artery territories.
     
    This case report stresses that for patients with brachial diplegia with normal supracervical findings, an atypical cervical cord INFARCTIOn should be considered even if the patient is comatose and has normal inferior extremities.
      

  • MG Edward, P Sampath, JA Williams et al (Univ of North Carolina, Chapel Hill; Johns Hopkins Med Institutions, Baltimore, Md).
      Local Delivery of Chemotherapy Prolongs Survival in Experimental Brain Metastases from Breast Carcinoma.
      Neurosurgery 43: 1185-1193, 1998.
        
    Local delivery of carmustine with biodegradable polymers can significantly prolong survival in a murine model of intracranial metastatic breast cancer. The incidence of local recurrence of breast cancer metastases with minimal morbidity may be decreased with surgical resection and placement of carmustine polymers into the resection cavity. 
        

 

 

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