If these defects are not repaired effectively, they may lead to meningoneural adhesions, greater scar tissue formation in the epidural region, pachymeningitis
or soft tissue infections, cerebrospinal fluid (CSF) leaks or collections, decreased wound healing, neural herniations, tethered cord and pseudomeningocele development, and also to neurological deficits as a consequence .
IgG4-related hypertrophic pachymeningitis
can cause localized or diffuse thickening of the dura mater (66).
The typical clinical features of OMAAV, recently proposed by the Japan Otological Society, are the following: (1) intractable otitis media with effusion or granulation that does not respond to antibiotics or insertion of a tympanic ventilation tube; (2) gradual hearing loss (in most cases) due to effusion and granulation in the middle ear, followed by sudden, progressive hearing loss within 2 months; (3) the MPO- or PR3-ANCA positivity (in most cases); (4) facial palsy and hypertrophic pachymeningitis
Wallace et al, (2) retrospectively examined 14 cases of pachymeningitis
at their institution over 25 years and found that IgG4-RD accounted for four of those cases or 66% of previously labeled idiopathic cases.
Other than TBM with leptomeningeal involvement, CNS tuberculosis can present in several ways such as intracranial tuberculomas, tuberculous pachymeningitis
, and spinal tuberculous arachnoiditis.
The main organs involved are the lymph nodes, salivary glands, lacrimal glands, and pancreas [4-10], which is very similar to our reported cases except the second case which had IgG4-related pachymeningitis
which is an extremely rare finding in our literature review.
Fetal autopsy showed a cutaneous erythematous papular disseminated rash, widespread edema (stronger in the limbs), ascites, hydrothorax, internal obstructed hydrocephalus, ischemichaemorrhagic brain injury with disseminated lesions, productive pachymeningitis
with dystrophic calcification, hepatomegaly, acute hepatic and splenic hematopoiesis, productive bilateral peripyelitis, pronounced glomerulus-poiesis, and involution of the thymus.
Moreover, there is also evidence of a case of IgG4-related hypertrophic pachymeningitis
of the posterior cranial fossa in a 52-year-old man presenting with vertigo, moderate bilateral hearing loss, tinnitus, and blurred vision.
However, due to lack of the typical cerebrospinal fluid profile (a predominance of lymphocytes, low glucose, and elevated protein) and MRI signs (tuberculomas, hydrocephalus, infarction, pachymeningitis
, etc.), TBM was excluded subsequently.