scholarly journals Factor VIII prophylactic therapy reduces neurological complications in patients with Hemophilia A

2021 ◽  
Author(s):  
José Marcelino Aragão FERNANDES ◽  
Francisco de Assis Aquino GONDIM ◽  
Antônia Maria Negreiro DIAS ◽  
Rosângela de Albuquerque RIBEIRO ◽  
Luiz Ivando Pires FERREIRA FILHO ◽  
...  
Keyword(s):  
Nervous System ◽  
Factor Viii ◽  
Peripheral Nervous System ◽  
Intracranial Hemorrhage ◽  
Prophylactic Treatment ◽  
Hemophilia A ◽  
Neurological Complications ◽  
Prophylactic Therapy ◽  
First Report ◽  
Before And After

ABSTRACT Background: Bleeding in hemophiliacs can cause complications in the central and peripheral nervous system (CNS and PNS). The incidence of intracranial hemorrhage has reduced after the introduction of prophylactic treatment with factor VIII or IX, but the benefits of this therapy have not yet been evaluated on PNS complications. Objective: The aim of this study was to determine the prevalence of neurological complications in hemophiliacs and verify the effect of prophylactic therapy in these patients, including PNS disorders. Methods: We retrospectively evaluated the prevalence of CNS and PNS disorders caused by bleeding in hemophiliacs seen at the Hemocentro Regional Norte, Ceará, Brazil, from 1992 to 2018, and we compared the incidence in different periods (before and after the introduction of prophylactic treatment in 2011). Results: Of 75 hemophilia A patients evaluated (4.61/100.000 population), 13.3% (n=10) had either CNS (n=5) or PNS (n=5) disorders secondary to bleeding. Patients submitted to factor VIII replacement prophylactic therapy were less likely to have CNS events: from 1992 to 2011, 5 of 63 patients had CNS disease, while from 2011 to 2018, there were no new cases (p=0.0181). From 2011 to 2018, 5 PNS events occurred in patients without prophylactic therapy, whereas none occurred in those covered by prophylactic therapy (5/20 versus 0/29, p=0.0081). Conclusions: The prevalence of neurological complications in hemophiliacs in our cohort is similar to other studies. Similar to CNS, prophylactic therapy also reduces the risk of PNS complications. This is the first report in the literature showing this benefit.

Nervous system

2013 ◽  
pp. 140-145
Author(s):  
Andrew Graham ◽  
Clare Galton
Keyword(s):  
Nervous System ◽  
Peripheral Nervous System ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Neurological Complications ◽  
Neurological Involvement ◽  
Disease Modifying Therapy ◽  
Entrapment Neuropathies ◽  
System P ◽  
Tunnel Syndrome

Rheumatological conditions may be complicated by a variety of both central and peripheral nervous system disorders. Common complications such as entrapment neuropathies are familiar to rheumatologists but accurate diagnosis of less common neurological disorders may be challenging; careful clinical reasoning is essential, supplemented where necessary by imaging, neurophysiology, and other special investigations including cerebrospinal fluid examination. Complications vary according to the nature of the background condition. In rheumatoid arthritis, neurological involvement is typically related to the mechanical consequences of advancing disease; most commonly, entrapment neuropathies such as carpal tunnel syndrome and cervical myelopathy due to atlantoaxial subluxation. By contrast, neurological involvement in systemic lupus erythematosus (SLE) tends to occur earlier in the disease course, with a much wider range of manifestations. The management of stroke or seizures in SLE is not necessarily any different from that in the general population, unless complicated by the antiphospholipid syndrome. However, less common neurological syndromes may demand more specific investigation and treatment. For example, longitudinally extensive transverse myelitis and recurrent optic neuritis (neuromyelitis optica, or Devic’s disease) is frequently associated with antibodies to aquaporin-4, and is highly likely to relapse unless treated vigorously with humoral immunosuppression. Nervous system involvement in vasculitis is common. Finally, not all neurological disorder in rheumatological disease is necessarily due to the underlying condition; neurological complications of disease-modifying therapy are increasingly recognized, in particular central and peripheral nervous system demyelination associated with TNF-α‎‎ inhibitors.

scholarly journals Effects of Spontaneous Chronic Hypoglycemia on Central and Peripheral Nervous System in Insulinoma Patients before and after Surgery: A Neurophysiological Follow-Up

1997 ◽  
Vol 82 (5) ◽  
pp. 1447-1451 ◽  
Author(s):  
Giuseppe Pozzessere ◽  
Elvira Valle ◽  
Carmelo D’Alessio ◽  
Giampiero Soldati ◽  
Francesco Pierelli ◽  
...  
Keyword(s):  
Nervous System ◽  
Peripheral Nervous System ◽  
Before And After

Rheumatoid Arthritis

2013 ◽  
Author(s):  
Aaron E. Miller ◽  
Teresa M. DeAngelis
Keyword(s):  
Rheumatoid Arthritis ◽  
Nervous System ◽  
Peripheral Nervous System ◽  
Inflammatory Disease ◽  
Neurological Complications ◽  
Neurologic Complications ◽  
Neurological Sequelae ◽  
Systemic Inflammatory Disease

Rheumatoid arthritis (RA) is a systemic inflammatory disease that is characterized principally by a polyarthritis, but can result in several neurologic complications involving both the central and peripheral nervous system. In addition, several immunotherapies used to treat RA have been associated with neurological complications. In this chapter, we review the characteristic neurological sequelae of RA as well as the possible neurological consequences of its therapeutic regimens.

scholarly journals Purification and characterization of factor VIII 372-Cys: a hypofunctional cofactor from a patient with moderately severe hemophilia A

Blood ◽  
1990 ◽  
Vol 75 (8) ◽  
pp. 1664-1672 ◽  
Author(s):  
DP O'Brien ◽  
JK Pattinson ◽  
EG Tuddenham
Keyword(s):  
Factor Viii ◽  
Heavy Chain ◽  
Time Course ◽  
Hemophilia A ◽  
Severe Hemophilia ◽  
Thrombin Cleavage ◽  
Before And After ◽  
Functional Defect ◽  
Thrombin Activation

We have purified factor VIII from a patient with moderately severe hemophilia A (FVIII, 4 U/dL; FVIII:Ag, 110 U/dL) and subjected the protein to Western blot analysis after time course activation with thrombin. The cross reacting material-positive (CRM+) FVIII has the normal distribution of heavy and light chains before thrombin activation, and, after incubation with the enzyme, appropriate cleavages are made at positions 740 and 1689. However, the normal thrombin cleavage at position 372 in the heavy chain of this molecule does not occur. This result is consistent with the demonstration in the patient's leukocyte DNA of a C to T transition in codon 372, leading to the substitution of a cysteine for an arginine residue at the heavy chain internal cleavage site. The severely impaired functional activity of this molecule confirms that the heavy chain of FVIII must be proteolysed in order to effect full cofactor activation in vivo. However, a threefold activation was detected when this protein was incubated with thrombin. No evidence of thrombin-mediated cleavage at position 336 in the heavy chain was detected, in contrast to the variant recombinant B domainless-molecule, FVIII 372-Ile, described by Pittman and Kaufman (Proc Natl Acad Sci USA 85:2429, 1988). Using gel permeation studies of the FVIII/von Willebrand factor (vWF) complex before and after thrombin activation, we have demonstrated that the 40 Kd A2 domain of wild type FVIII dissociates from vWF after cleavage by the enzyme. In contrast, incomplete dissociation was detected in the case of FVIII 372-Cys. We conclude that the functional defect in FVIII 372-Cys is a consequence of the resistance to proteolysis of the internal scissile bond in the heavy chain.

Nervous system

2013 ◽  
Author(s):  
Andrew Graham ◽  
Clare Galton
Keyword(s):  
Nervous System ◽  
Peripheral Nervous System ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Neurological Complications ◽  
Neurological Involvement ◽  
Disease Modifying Therapy ◽  
Entrapment Neuropathies ◽  
System P ◽  
System Disorder

Rheumatological conditions may be complicated by a variety of both central and peripheral nervous system disorder. Common complications such as entrapment neuropathies are familiar to rheumatologists but accurate diagnosis of less common neurological disorders may be challenging; careful clinical reasoning is essential, supplemented where necessary by imaging, neurophysiology, and other special investigations including cerebrospinal fluid examination. Complications vary according to the nature of background condition. In rheumatoid arthritis, neurological involvement is typically related to the mechanical consequences of advancing disease; the commonest complications are carpal tunnel syndrome and cervical myelopathy due to atlantoaxial subluxation. By contrast, neurological involvement in systemic lupus erythematosus (SLE) tends to occur earlier in the disease course, with a much wider range of manifestations. The management of stroke or seizures in SLE is not necessarily any different from that in the general population, unless complicated by the antiphospholipid syndrome. However, less common neurological syndromes may demand more specific investigation and treatment. For example, longitudinally extensive transverse myelitis and recurrent optic neuritis (neuromyelitis optica, or Devic's disease) is frequently associated with antibodies to aquaporin-4, and is highly likely to relapse unless treated vigorously with humoral immunosuppression. Nervous system involvement in vasculitis is common. Finally, not all neurological disorder in rheumatological disease is necessarily due to the underlying condition; neurological complications of disease-modifying therapy are increasingly recognized, in particular central and peripheral nervous system demyelination associated with TNF-α‎ inhibitors.

scholarly journals COVID-19 Infection and Neurological Complications: Present Findings and Future Predictions

2020 ◽  
Vol 54 (5) ◽  
pp. 364-369 ◽  
Author(s):  
Ettore Beghi ◽  
Valery Feigin ◽  
Valeria Caso ◽  
Paola Santalucia ◽  
Giancarlo Logroscino
Keyword(s):  
Nervous System ◽  
Peripheral Nervous System ◽  
Viral Infections ◽  
Neurological Diseases ◽  
Neurological Complications ◽  
Influenza Viruses ◽  
Surveillance Systems ◽  
Care Needs ◽  
The Past ◽  
Immune Mediated

The present outbreak caused by SARS-CoV-2, an influenza virus with neurotropic potential, presents with neurological manifestations in a large proportion of the affected individuals. Disorders of the central and peripheral nervous system are all present, while stroke, ataxia, seizures, and depressed level of consciousness are more common in severely affected patients. People with these severe complications are most likely elderly with medical comorbidities, especially hypertension and other vascular risk factors. However, postinfectious complications are also expected. Neurological disorders as sequelae of influenza viruses have been repeatedly documented in the past and include symptoms, signs, and diseases occurring during the acute phase and, not rarely, during follow-up. Postinfectious neurological complications are the result of the activation of immune mechanisms and can explain the insurgence of immune-mediated diseases, including the Guillain-Barré syndrome and other diseases of the central and peripheral nervous system that in the past occurred as complications of viral infections and occasionally with vaccines. For these reasons, the present outbreak calls for the introduction of surveillance systems to monitor changes in the frequency of several immune-mediated neurological diseases. These changes will determine a reorganization of the measures apt to describe the interaction between the virus, the environment, and the host in areas of different dimensions, from local communities to regions with several millions of inhabitants. The public health system, mainly primary care, needs to be strengthened to ensure that research and development efforts are directed toward right needs and directions. To cope with the present pandemic, better collaboration is required between international organizations along with more research funding, and tools in order to detect, treat, and prevent future epidemics.

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