- Email: [email protected]
3-25-07 Miller Fisher-Guillain-Barré overlap syndrome with brainstem pathological MRI findings
Injlammatory
& Immunological
episode the patient developed a new quadrfplegia event along with glove and sock disesthesiae and hand feet cyanosis and sweating. 2 years later after begins off steroids patient had anew onset of quadrtplegia, propioceptive, motor end sensory dispairment. Paraclinics were performed: Toxics, GOT, GPT, Bone gammagrafy, several x-rays, tumor markers, alkaline phosphatase. C3, C4, ANA, LE cells, Anti-DNA, protein electrophoresis; all of them negative. Biopsy of sural nerve was performed with negatiie results. Neuroconduction studies showed severe compromise of axons and myelin. Patient was treated with Carbamazepine, Amitryptiline, Oligoelements, physical therapy and steroids, In summary we have a 52 years old male with 3 episodes of Polineuropathy with vegetative, sensory and motor distal compromise; who responded to steroids.
3-25-03
E. Dias-Tosta, Brasilia
Acute flaccid palsies (AFP) and Guiiiain-Barre syndrome (GBS) in a population less than 15 years old in Brazil C. Santos-Kuckelhaus.
&spiral
de Base do Distrito Federal,
To know the impact of the GBS in the population less than 15 years old, after the erradication of poliomyelitis. Data bank from the prooram of epidemioloaical surveillance of AFP from the Fundacao National de Saljde were analysed between 1990-l 996. From 3567 notifiittons of AFP there were 1616 GBS. GBS yearly incidence rates is 0.42 to 0.60 cases per 100,000 population, with occasional higher incidences up to 1.49 in the north region of Brazil. No consistent seasonal variation existed. Weakness at inclusion were mild in 2%, medium in 43% and severe in 55%. Sixty days after 46% were normal, 36% still had motor signs, death occurred in 7% and 7% unknown. Death rate vanes from 3% in southeast region to 9% in northeast. Nowadays GBS is the most frequent cause of AFP in this population. In spite of the seventy of this disease being similar in the different regions, the outcome varies according to origin of the cases, possibly reflecting the economical condiiions in those places.
3-25-04
increased levels of beta -trace protein and intrathecai synthesis of IgG subclasses in Cuban Epidemic Neuropathy
A.J. Dorta-Contreras ‘, H. Reiber2, E. Martinez-Torres 3. ’ /mmuno@y Lab Hospite/ pedietdco San Miguel, Ciudad Habana, Cuba, 2 Neurochemistry Lab Neutviogy Clinic, Goettingen, Germany 3 Hospital Pedi&ico Juan Manuel Mzirquez, Ciudad Habana, Cuba Cuban Epidemic Neumpathy (CEN) is an emergent disease. Its etiology remains unknown. The pediatric population was the less affected one. Three pediatric patients with CEN were studied. Cerebmspinal fluid (CSF) and sera were simultaneously obtained. Albumin and IgG subclasses were quantified by immunodiffusion. Beta-trace protein (BTP) was determined by nephelometry. Local synthesis of IgG subclasses was calculated by Reiber’s quotient diagram. Increased levels of BTP in comparison with a cuban pediatric control group were observed. All the group had local synthesis of IgG subclasses. More than 60% of lgG2, IgGs, and IgG4 found in CSF was locally synthesized and between 60-60% of CSF IgGr content was intrathecally made. The results indicates neumn damage and a probable role of the local immune system in this disease.
3-25-05
immunopathoiogkai diabetic amyotrophy
findings
in a patient with
J. Drulovib, S. Apostolski, M. Doiic, S. Doiic, N. Stojsavljevic, Z. Levi& Institute of Neurofog~ Clinical Center of Setia, School of Medicine, University of Belgrade, &gosiavia It has been implicated that immune mechanisms may be involved in the pathogenesis of some varieties of diabetic neuropathies. We report findings of immunohistochemical study of the sural nerve in a patient with severe diabetic amyotrophy. A 70-yearold male with non-insulin-dependent diabetes mellitus presented with painful, asymmetrical, lower extremities weakness which gradually worsened over a 15month period. Nerve conduction studies revealed evidence of predominantly axonal and motor polyneuropathy. Laboratory analyses showed elevated erythrocyte sedimentation rate (130/h), increased cerebrospinal fluid pmtein level (0.76 g/L) and cell content (16 lymphocytes/mm3). M-protein, anti-GM1 and anti-MAG antibodies were negative. Patient underwent sure1 nerve biopsy. lmmunofluorescence examination of the sural nerve revealed IgG deposits along the myelin sheaths in over 70% of the examined nerve fibers. Long-term steroid therapy produced marked improvement. This case may suggest a role for humoral immunity in diabetic amyotrophy.
Neuropathies
3-25-06
s177
1 Clinical studies of poiyneuropathy associated with monocionai gammopathy with anti MAG/SGPG antibody in Japan
T. Saito, T. Nukazawa, Y. Clgino, M. Ogino, H. Ito, H. Kowa. Department Neurolog)! Kitasato University East Hospital, Kanagawa, Japan
of
We studied the clinical features, and electmphysiological and histologic studies of biopsied peripheral nerves and serum antibodies to peripheral nerve tissue in 26 patients with IgM monoclonal gammopathy. Nineteen of 20 patients with IgM monoclonal gammopathies undetermined significance (or nonmalignant IgM monoclonal gammopathy) had high tiieres of myelin-associated glycoprotein (MAG) IgM antibodies by ELISA or Immunoblots. All of them were also detected IgM antibodies to sulfated glucuronyl paragloboside (SGPG). Two of 26 patients had serum IgM anti-SGPG anttbodies but not IgM anti-MAG antibodies. Main clinical features in 26 patients were slowly progressive ataxic sensory neuropathy with minimum motor deficits. There were no differences of clinical features between MAG-reactive and MAO-non-reactive patients. Sensory action potentials were not obtained in the nerves of the lower extremities in most cases. Nerve biopsy specimens in 16 patients disclosed demyelination with or without axonal degeneration. Electron microscopy showed increased separation of some major dense lines in the outer hatf of the myelin sheath of myelinated fibers in seven of 10 patients. The effects of plasmapheresis or prednisdone were transient. Further therapeutic trial is needed. There were no clinical differences between in Japan and Western countries in patients with polyneuropathy associated with IgM monoclonal gammopathy.
3-25-07
Miller Fisher-Guiiiain-Barre overlap syndrome brainstem pathological MM findings
R.J. Giannaula, M.B. Seijas, D.A. Ndriez, J.C. Maiques, E.E. Castillo. Neurology. Hospital Espafiol, Buenos Aires, Reptiblica Argentina
with Dept. of
Whether Miller Fisher syndrome is secondary to a central lesion or a variant of the Guillain-Barre syndrome is still matter of conjecture. MRI have proven positive in some cases but not in others showing evidence of peripheral nervous system involvement. Objective: To report the association between Guillain-Barre and Miller Fisher’s syndrome as well as central lesions in the latter. Patkntdhkthods: A P&year-dd man developed ophthalmoplegia, ataxia, quadriparesis and left facial palsy. Deep tendon reflexes were absent and no sensory impairment was detected. MRI showed hyperintense T2-weighted images located in the pons, medulla, left inferior cerebellar peduncle and perfaqueductal gray matter. A 46year-old man was admitted with dysphagia, dysarthria, ataxia, a right VI th cranial nerve palsy and quadrfparesis. Deep tendon reflexes were absent and no sensory impairment was detected. MRI showed hypertntense T2-weighted images located in the pans, medulla and both middle cerebellar peduncles. Electrophysiological studies in both patients showed evidence of a demyelinating neumpathy while CSF analysis showed a high protein content. Conclusions: Our patients had a Miller Fisher syndrome with both peripheral and CNS damage, suggesting that Miller Fisher and Guillain-Barre syndromes may share a common pathophysiotogical mechanism.
3-25-08
Eiectrophysioiogicai follow-up of 45 patients with chronic inflammatory demyeiinating poiyradicuioneuropathy (CIDP)
C. Ciano, C. Martotti, E. Palazzini, D. Pareyson, ktituto Nazionale Neuro/ogico Y: Besta”, Wan,
E. Fallica, A. Sghirlanzoni. Italy
We evaluated 46 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to better define electmphysiological features and course. Thirty-four patients had idiopathic CIDP, 11 had CIDP associated with monoclonal gammopathy of undetermined significance (CIDP-MGUS). The patients were followed up electrophysiologically for 2 to 12 years (mean 4.4). Electrophysiological investigations were performed using standard techniques. Strict electrcdiagnostii criteria for demyelination without superimposed axonal degeneration were fulfilled by 36% of patients. In the remaining cases, nerve conduction studies and electromyography indicated combined axonal degeneration and demyelination. Conduction slowing was relatively uniform, both along individual nerves and between different nerves. The most common presentation was a symmetric sensorimotor neuropathy. CIDP-MGUS had chiefly sensory deficits. Responses to treatment and the seventy of the neuropathy varied greatly between patients with similar disease duration. After 2 years, demyelination without axonal loss was still observed in only 24% of patients. Conduction velocity changes paralleled the clinical course. Sixty-one % of patients with an initially predominantly demyeliwting polyneuropathy had a good outcome at last follow-up examination. On the other hand, only 33% of patients with axonal loss had a favorable course.
& Immunological
episode the patient developed a new quadrfplegia event along with glove and sock disesthesiae and hand feet cyanosis and sweating. 2 years later after begins off steroids patient had anew onset of quadrtplegia, propioceptive, motor end sensory dispairment. Paraclinics were performed: Toxics, GOT, GPT, Bone gammagrafy, several x-rays, tumor markers, alkaline phosphatase. C3, C4, ANA, LE cells, Anti-DNA, protein electrophoresis; all of them negative. Biopsy of sural nerve was performed with negatiie results. Neuroconduction studies showed severe compromise of axons and myelin. Patient was treated with Carbamazepine, Amitryptiline, Oligoelements, physical therapy and steroids, In summary we have a 52 years old male with 3 episodes of Polineuropathy with vegetative, sensory and motor distal compromise; who responded to steroids.
3-25-03
E. Dias-Tosta, Brasilia
Acute flaccid palsies (AFP) and Guiiiain-Barre syndrome (GBS) in a population less than 15 years old in Brazil C. Santos-Kuckelhaus.
&spiral
de Base do Distrito Federal,
To know the impact of the GBS in the population less than 15 years old, after the erradication of poliomyelitis. Data bank from the prooram of epidemioloaical surveillance of AFP from the Fundacao National de Saljde were analysed between 1990-l 996. From 3567 notifiittons of AFP there were 1616 GBS. GBS yearly incidence rates is 0.42 to 0.60 cases per 100,000 population, with occasional higher incidences up to 1.49 in the north region of Brazil. No consistent seasonal variation existed. Weakness at inclusion were mild in 2%, medium in 43% and severe in 55%. Sixty days after 46% were normal, 36% still had motor signs, death occurred in 7% and 7% unknown. Death rate vanes from 3% in southeast region to 9% in northeast. Nowadays GBS is the most frequent cause of AFP in this population. In spite of the seventy of this disease being similar in the different regions, the outcome varies according to origin of the cases, possibly reflecting the economical condiiions in those places.
3-25-04
increased levels of beta -trace protein and intrathecai synthesis of IgG subclasses in Cuban Epidemic Neuropathy
A.J. Dorta-Contreras ‘, H. Reiber2, E. Martinez-Torres 3. ’ /mmuno@y Lab Hospite/ pedietdco San Miguel, Ciudad Habana, Cuba, 2 Neurochemistry Lab Neutviogy Clinic, Goettingen, Germany 3 Hospital Pedi&ico Juan Manuel Mzirquez, Ciudad Habana, Cuba Cuban Epidemic Neumpathy (CEN) is an emergent disease. Its etiology remains unknown. The pediatric population was the less affected one. Three pediatric patients with CEN were studied. Cerebmspinal fluid (CSF) and sera were simultaneously obtained. Albumin and IgG subclasses were quantified by immunodiffusion. Beta-trace protein (BTP) was determined by nephelometry. Local synthesis of IgG subclasses was calculated by Reiber’s quotient diagram. Increased levels of BTP in comparison with a cuban pediatric control group were observed. All the group had local synthesis of IgG subclasses. More than 60% of lgG2, IgGs, and IgG4 found in CSF was locally synthesized and between 60-60% of CSF IgGr content was intrathecally made. The results indicates neumn damage and a probable role of the local immune system in this disease.
3-25-05
immunopathoiogkai diabetic amyotrophy
findings
in a patient with
J. Drulovib, S. Apostolski, M. Doiic, S. Doiic, N. Stojsavljevic, Z. Levi& Institute of Neurofog~ Clinical Center of Setia, School of Medicine, University of Belgrade, &gosiavia It has been implicated that immune mechanisms may be involved in the pathogenesis of some varieties of diabetic neuropathies. We report findings of immunohistochemical study of the sural nerve in a patient with severe diabetic amyotrophy. A 70-yearold male with non-insulin-dependent diabetes mellitus presented with painful, asymmetrical, lower extremities weakness which gradually worsened over a 15month period. Nerve conduction studies revealed evidence of predominantly axonal and motor polyneuropathy. Laboratory analyses showed elevated erythrocyte sedimentation rate (130/h), increased cerebrospinal fluid pmtein level (0.76 g/L) and cell content (16 lymphocytes/mm3). M-protein, anti-GM1 and anti-MAG antibodies were negative. Patient underwent sure1 nerve biopsy. lmmunofluorescence examination of the sural nerve revealed IgG deposits along the myelin sheaths in over 70% of the examined nerve fibers. Long-term steroid therapy produced marked improvement. This case may suggest a role for humoral immunity in diabetic amyotrophy.
Neuropathies
3-25-06
s177
1 Clinical studies of poiyneuropathy associated with monocionai gammopathy with anti MAG/SGPG antibody in Japan
T. Saito, T. Nukazawa, Y. Clgino, M. Ogino, H. Ito, H. Kowa. Department Neurolog)! Kitasato University East Hospital, Kanagawa, Japan
of
We studied the clinical features, and electmphysiological and histologic studies of biopsied peripheral nerves and serum antibodies to peripheral nerve tissue in 26 patients with IgM monoclonal gammopathy. Nineteen of 20 patients with IgM monoclonal gammopathies undetermined significance (or nonmalignant IgM monoclonal gammopathy) had high tiieres of myelin-associated glycoprotein (MAG) IgM antibodies by ELISA or Immunoblots. All of them were also detected IgM antibodies to sulfated glucuronyl paragloboside (SGPG). Two of 26 patients had serum IgM anti-SGPG anttbodies but not IgM anti-MAG antibodies. Main clinical features in 26 patients were slowly progressive ataxic sensory neuropathy with minimum motor deficits. There were no differences of clinical features between MAG-reactive and MAO-non-reactive patients. Sensory action potentials were not obtained in the nerves of the lower extremities in most cases. Nerve biopsy specimens in 16 patients disclosed demyelination with or without axonal degeneration. Electron microscopy showed increased separation of some major dense lines in the outer hatf of the myelin sheath of myelinated fibers in seven of 10 patients. The effects of plasmapheresis or prednisdone were transient. Further therapeutic trial is needed. There were no clinical differences between in Japan and Western countries in patients with polyneuropathy associated with IgM monoclonal gammopathy.
3-25-07
Miller Fisher-Guiiiain-Barre overlap syndrome brainstem pathological MM findings
R.J. Giannaula, M.B. Seijas, D.A. Ndriez, J.C. Maiques, E.E. Castillo. Neurology. Hospital Espafiol, Buenos Aires, Reptiblica Argentina
with Dept. of
Whether Miller Fisher syndrome is secondary to a central lesion or a variant of the Guillain-Barre syndrome is still matter of conjecture. MRI have proven positive in some cases but not in others showing evidence of peripheral nervous system involvement. Objective: To report the association between Guillain-Barre and Miller Fisher’s syndrome as well as central lesions in the latter. Patkntdhkthods: A P&year-dd man developed ophthalmoplegia, ataxia, quadriparesis and left facial palsy. Deep tendon reflexes were absent and no sensory impairment was detected. MRI showed hyperintense T2-weighted images located in the pons, medulla, left inferior cerebellar peduncle and perfaqueductal gray matter. A 46year-old man was admitted with dysphagia, dysarthria, ataxia, a right VI th cranial nerve palsy and quadrfparesis. Deep tendon reflexes were absent and no sensory impairment was detected. MRI showed hypertntense T2-weighted images located in the pans, medulla and both middle cerebellar peduncles. Electrophysiological studies in both patients showed evidence of a demyelinating neumpathy while CSF analysis showed a high protein content. Conclusions: Our patients had a Miller Fisher syndrome with both peripheral and CNS damage, suggesting that Miller Fisher and Guillain-Barre syndromes may share a common pathophysiotogical mechanism.
3-25-08
Eiectrophysioiogicai follow-up of 45 patients with chronic inflammatory demyeiinating poiyradicuioneuropathy (CIDP)
C. Ciano, C. Martotti, E. Palazzini, D. Pareyson, ktituto Nazionale Neuro/ogico Y: Besta”, Wan,
E. Fallica, A. Sghirlanzoni. Italy
We evaluated 46 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to better define electmphysiological features and course. Thirty-four patients had idiopathic CIDP, 11 had CIDP associated with monoclonal gammopathy of undetermined significance (CIDP-MGUS). The patients were followed up electrophysiologically for 2 to 12 years (mean 4.4). Electrophysiological investigations were performed using standard techniques. Strict electrcdiagnostii criteria for demyelination without superimposed axonal degeneration were fulfilled by 36% of patients. In the remaining cases, nerve conduction studies and electromyography indicated combined axonal degeneration and demyelination. Conduction slowing was relatively uniform, both along individual nerves and between different nerves. The most common presentation was a symmetric sensorimotor neuropathy. CIDP-MGUS had chiefly sensory deficits. Responses to treatment and the seventy of the neuropathy varied greatly between patients with similar disease duration. After 2 years, demyelination without axonal loss was still observed in only 24% of patients. Conduction velocity changes paralleled the clinical course. Sixty-one % of patients with an initially predominantly demyeliwting polyneuropathy had a good outcome at last follow-up examination. On the other hand, only 33% of patients with axonal loss had a favorable course.