Neurologic Involvement in Primary Immunodeficiency Disorders

2018 ◽  
Vol 33 (5) ◽  
pp. 320-328 ◽  
Author(s):  
Mirac Yildirim ◽  
Deniz Cagdas Ayvaz ◽  
Bahadir Konuskan ◽  
Rahsan Gocmen ◽  
Ilhan Tezcan ◽  
...  
Keyword(s):  
Immune Deficiency ◽  
Central Nervous System ◽  
Nervous System ◽  
Vascular Complication ◽  
Primary Immune Deficiency ◽  
Initial Manifestation ◽  
Neurologic Manifestation ◽  
Neurologic Involvement ◽  
Cognitive Delay ◽  
Central Nervous System Malformation

The nervous system may be affected in primary immune deficiency (PID) syndromes through infectious, autoimmune, neoplastic mechanisms, or as a primary feature of the syndrome. However certain neurologic problems do not conform to these etiopathogenetic groups. We retrospectively examined PID patients seen in neurology consultation between 2014 and 2017 in order to determine the spectrum of nervous system involvement. Among patients with confirmed neurologic problems (n = 35), common manifestations were encephalopathy and global developmental/cognitive delay. In 13 (37%) instances, the neurologic signs had no apparent relation with a treatment-related, infectious, or vascular complication and were considered as primary findings: acquired microcephaly, central nervous system malformation, or peripheral neuropathy. The diagnosis of PID was made after, and based on, the neurologic manifestation in 6 of 35 (17%) patients. Neurologic presentation may constitute the initial manifestation in some types of primary immune deficiency.

scholarly journals Neurologic manifestation in children parenterally infected by AIDS

2001 ◽  
Vol 82 (5) ◽  
pp. 356-358
Author(s):  
T. Yu. Burdaeva ◽  
N. V. Bobrysheva
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Manifestations ◽  
Brain Biopsy ◽  
High Rate ◽  
Neurologic Manifestation ◽  
Progressive Encephalopathy ◽  
Neuropsychologic Testing ◽  
Magnet Resonance ◽  
Standard Tests

The rate and spectrum of central nervous system lesion in children parenterally infected by AIDS the rate of clinical manifestations of lesions, life duration of such children are established. The differential diagnosis of nervous system lesion is made difficult due to the high rate of combining various symptoms in one patient, hence there are the complications of selecting the treatment tactics. Tomography, nuclear magnet resonance, brain biopsy should be used, neuropsychologic testing with standard tests should be performed to reveal the early signs of progressive encephalopathy.

Relapsing myelopathy as the initial manifestation of primary central nervous system angiitis

2007 ◽  
Vol 55 (4) ◽  
pp. 425
Author(s):  
VV Ashraf ◽  
MathewM John ◽  
KG Ramakrishnan ◽  
Anita Mahadevan
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Initial Manifestation

A Diagnostic Pointer to Adult Metachromatic Leucodystrophy

1980 ◽  
Vol 137 (2) ◽  
pp. 186-187 ◽  
Author(s):  
John A. O. Besson
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Autosomal Recessive ◽  
Demyelinating Disease ◽  
Initial Manifestation ◽  
The Central Nervous System ◽  
Metachromatic Leucodystrophy ◽  
Arylsulphatase A ◽  
Recessive Defect

Adult metachromatic leucodystrophy (MLD) is a rare demyelinating disease of the central nervous system caused by a genetic autosomal recessive defect and mediated through a deficiency in the enzyme arylsulphatase A (Peiffer, 1970). The initial manifestation may take the form of symptoms suggestive of schizophrenia or dementia (Sourander et al, 1962; Austin et al, 1968).

Malformation of the Brain, Skull, and Spine

2021 ◽  
pp. 1057-1070
Author(s):  
Lily C. Wong-Kisiel
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Developmental Delay ◽  
Abnormal Development ◽  
Gene Expressions ◽  
Common Cause ◽  
Genetic Causes ◽  
The Central Nervous System ◽  
The Brain ◽  
Central Nervous System Malformation

Abnormal development of the central nervous system is a common cause of developmental delay and epilepsy. An understanding of central nervous system malformation begins with an overview of normal embryology. Genetic advances in embryogenesis have unfolded a complex orchestration of gene expressions in place of the traditional developmental epochs (induction, neurulation, proliferation, migration, organization, synaptogenesis, and myelination). Causes of malformation of the central nervous system are multifactorial. Genetic causes, vitamin excess or deficiency, infections, or teratogens any time during pregnancy may disturb the preprogrammed mechanisms.

Central nervous system aquaporin-4 autoimmunity presenting with an isolated cerebral abnormality

2012 ◽  
Vol 18 (9) ◽  
pp. 1340-1343 ◽  
Author(s):  
Su-Hyun Kim ◽  
Woojun Kim ◽  
Myeong-Cherl Kook ◽  
Eun-kyung Hong ◽  
Ho Jin Kim
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Optic Neuritis ◽  
Treatment Response ◽  
Comprehensive Evaluation ◽  
Cerebral Lesion ◽  
Cerebral Lesions ◽  
Initial Manifestation ◽  
Clinical Presentations ◽  
History Of

Advances in the understanding of central nervous system aquarporin-4 autoimmunity have promoted the recognition of diverse clinical presentations beyond the traditional view of neuromyelitis optica. We describe a patient who developed hemiparesis caused by an extensive cerebral lesion as an initial manifestation of central nervous system aquarporin-4 autoimmunity. Although the patient had no history of optic neuritis or myelitis, not only was serum anti-aquarporin-4 antibody positive, but an imaging, treatment response and histopathological features also revealed characteristic findings suggestive of central nervous system aquarporin-4 autoimmunity. The present case highlights the importance of a comprehensive evaluation for anti-aquarporin-4 antibody even in patients presenting with isolated cerebral lesions.

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