1 case of hypertrophy hard meningitis with a recurrence of headache after treatment

Hypertrophic hard meningitis (hypertrophiciciccial pachyhys, HCP) is a rare inflammatory hypertrophic disease characterized by epidural limitation or diffuse thickening, fibrosis as the main characteristic, the disease is hidden, the cause is complex, often manifested in clinical manifestations of chronic headache, epilepsy, mental abnormalities, etc., often can affect multiple groups of nerves The most commonly damaged cranial nerves are VIII, VI, IX to the brain nerves, and part of HCP is combined with hypertrophic spinal pachymeningitis (hypertrophic spinal pachymeningitis, HSP), known as hypertrophic ichymenitis (hypertrophic pachymeningitis, HP)Because of the diversity of HCP clinical performance, so clinical diagnosis is difficult, now will be admitted to our department of 1 case of HCP cases reported as follows1Case Informationpatient, male, 62 years old, was admitted to hospital on 9 July 2018 due to "one year of intermittent headache, six months aggravated by six months"Patients 1 year ago without obvious causes to start full head pain, sometimes for needle-like pain, sometimes for swelling pain, with the right ear hearing pain, intermittent double tinnitus, with insomnia, sleep difficulties, no nausea, vomiting, disregard for rotation, no fever, dizziness, unconscious loss, no limb numbness, headache each time can be self-alleviated for several hoursSix months ago the patient self-conscious headache aggravated, persisting, had been hospitalized in the hospital to a large dose of hormone shock treatment, the initial headache symptoms completely disappeared, about 3 months after the headache attack, after another hospitalization to give hormones and thiopental treatment, headache no significant improvement, for further diagnosis and treatment to my hospitaladmitted to the hospital physical examination: body temperature 36.5 degrees C, pulse 84 times / minute, breathing 20 times / minute, blood pressure 100/ 70mmHg (1mmHg - 0.133kPa), clear mind, smooth language, hearing loss of both ears, right ear hearing is very serious, double-eye ball no prominent, double diameter 3mm, photoreflection sensitive, eye activity full, no eye Seismic, double-sided forehead, nasal lip trench symmetry and other deep, tongue-in-the-middle, double-sided soft palate lift symmetry, hanging in the middle, pharynx reflection normal, limb muscle Vlevel, normal muscle tension, quadriplegic tendon reflection symmetric lead, bi-sided Babinski, Chaddock (-), the common movement normal, deep feeling normal, neck soft, Kassisted examination: electrocardiogram, liver and kidney function, blood sugar, myocardial spectrum, homocysteine did not see abnormalitiesCRP72.70mg/L, blood sink 75mm/h, PCT 0.05ng/mLCerebral spinal fluid and drug sensitivity, cerebrospinal fluid smear tuberculosis antibodies, cerebrospinal fluid ink staining are normalMale tumors are full yannedCerebral ridge fluid bright, pressure 123mmH2O, protein 91.0mg/dL, Pan's test positive, chlorine, glucose are normalThere is no obvious cellular composition in cerebrospinal fluid cytological stoicismCerebral spinal fluid immunology, cerebroprotein histology monitoring and analysis report: oligoclon region with negative, LgG index is high September 2, 2016 autoantibodies test: antinuclear antibody 1:320 positive, antinuclear antibody 1:1000 positive, anti-SSA-60kD antibody positive, anti-SSA-52kD antibody positive, anti-SSB antibody weak positive Antimyelin peroxidase antibody weak positive Serum anti-NMO antibody IgG negative Cerebral spinal fluid anti-NMO antibody IgG negative Immune 5: immunoglobulin Ig19.90g/L, IgM, IgA, C3, C4 are all normal Cytomegalovirus antibody IgG170U/mL, IgM5.70AU/mL There were no abnormalities in the hemodule protein chip and T-spot 25 August 2016 head MRI enhancement shows: (1) the right many chronic thickening, strengthening, considering the possibility of meningitis, do not exclude the cause of vascular lesions; On August 27, 2016, cranial mrA of the craniofacial arteries and intracranial venous MRV were not abnormal January 8, 2018 head MR enhancement: the right epidural is widely thickened, part of the empty butterfly saddle, the right nose armor is fat July 12, 2018 head MR enhanced MR tip: the cerebellum screen thickening, enhance obvious strengthening, the brain gap slightly widened, b-sinus, trans-sinus signal uneven, see Figure 1 Vestibular function: visual motion reflection abnormal, balance integration normal Chest CT: chronic bronchitis, emphysema, pulmonary follicle; EEG: Normal Epimenes biopsy pathology: under the mirror see fibrous connective tissue proliferation, part of the vascular wall glass-like degeneration or necrosis, interstitial intake to lymphocytes, plasma cells-based chronic inflammatory cells immersion, the area of the bureau stove see granuloma formation, in line with HCP, see Figure 2 Figure 1 Patient secrantic MRI on July 12, 2018 Note: A: T1-FFE-SAG-C; B:T1-FFE-COR-C; C:T1-FFE-TRA There is no obvious abnormal signal shadow in the essence of the brain, the right epidural enhanced see high signal indicates that the epidural is widely thickened, the cerebellum screen thickens, the enhancement is obviously strengthened, the brain trench, the brain is slightly wider, the ventricle, the brain pool is not large, the middle line structure is the patient's epidural biopsy (HE dying, x 100) Note: A large number of chronic inflammatory cells in dense fiber tissue dip
2 Discussion HP according to the etiology sub-specific and secondary, according to the affected site sub-HCP and HSP, the above-reported case is HCP At present, the etiology and pathogenesis of HCP are not clear, according to the etiology can be divided into isothal and secondary The etiology of idiopathic hypertrophic icic pachymeningitis (IHCP) is unknown, and autoimmune and immune disorders may play an important role in its development Secondary hypertrophic ici pachymeningitis (SHCP) is mostly associated with infectious diseases (bacteria, syphilis, fungi, bacillus, cysts), malignant tumors, hematologic diseases, lymphomas, etc in recent years, the literature reported that more and more SHCP secondary to some infectious diseases, pathogens through the middle ear, cete, sinus and fractured skull and other skull adjacent organ infection directly affected by the epidural is the main cause of SHCP disease, in addition, SHCP may coexist with intracranial tumors, POEMS syndrome, rheumatoid arthritis, regenerative anemia and so on Clinical analysis shows that HCP can occur in any age group, the ratio of men to women is about 3:1 The patient in this case was male Clinical manifestations are chronic headache, epilepsy, mental disorders, etc Most patients with headache as the main or first symptom, analysis of the cause of headache may be the meninges chronic inflammatory stimulation, fat imendoes compressed sinuses affect the reflux of the sinitus caused by intracranial hypertension The branch of the v.V cranial nerve is introduced into the pain center, and the resulting headache is located in the forehead, temporal and top areas of the ear The analysis of the patient's cerebrospinal fluid pressure is normal, considered with the thickening of the meninges of the chronic inflammatory stimulation imaging examination has guiding significance for the disease, head-reinforced MRI has important value for HCP diagnosis, enhanced scanning to diffuse reinforcement, but also can appear nodule-like reinforcement, if nodule-like reinforcement multi-hint prognosis is poor, lesions and reinforcement occurred in the probability of the occurrence of the site: cerebellum syllm, temporal, top epidural and restive mening The most characteristic imaging performance is: in the brain, cerebellum screen strengthening performance is "two-track" or "Benz", that is, the lesions of the epidural on both sides of the epicentre of the "track-like" reinforcement, the center for the line-like no-reinforcement zone In the ordinary flat sweep sequence, T1WI is equal, slightly lower signal, T2WI is a low signal, or no anomalies This patient complies with this change The diagnosis of HCP relies on a pathological examination Because there are fewer pathological tests in clinical practice, most patients can only make clinical diagnoses based on symptoms, signs and imaging In this case, the case followed by a pathological examination of the epidural after obtaining the patient's consent, the result was a nonspecific inflammatory change in the fat epidural If other special pathogens, such as tuberculosis, fungi, etc can be found in the course of medical examination, it provides a reliable basis for etiological diagnosis SHCP in the treatment of the original disease at the same time (e.g anti-tuberculosis, antifungal, antiviral, etc.), the use of high-dose hormone shock therapy (clinically more multi-use of meta-strong dragon 500 mg/d static drops), small doses to maintain treatment, in the process of glucocorticoid reduction of the disease recurrence or aggravation of the need to re-increase the dose of glucocorticoids or the use of glucocorticoid combined immunosuppressants treatment some scholars believe that for patients who are clinically diagnosed with IHCP, the treatment of glucocorticoids alone is easy to repeat, and it is necessary to add immunosuppressants in the hormone reduction process This case of the initial stage of the treatment of glucocorticoids is better, but because the patient after discharge from the hospital to stop the drug to cause a recurring headache attack, again to give metazi nylon impact treatment, and in the acetate pernipine tablet 60mg gradually reduced in the process of adding thiopental tablets 0.1g/d, the disease gradually improved, no recurrence the case of this article is male patients, clinical manifestations are mainly chronic whole headache, no complaints of visuals, co-help, swallowing, drinking water and other abnormalities, and the neurobody physical examination did not see obvious positive signs of cranial nerve damage, head-reinforced MRI did not see the physical damage to the brain, but there is a clear chronic thickening, strengthening of the right meninges, At the same time, the patient has a significant decline in hearing in the right ear, head MRI prompts the right nose armor hypertrophy, consider secretion of otitis media, and finally consider this patient may be caused by secondary ear-borne infection, check the cerebrospinal fluid LgG index is high, consider may not be a direct bacterial infection, may be autoimmune-related , the incidence of HCP is low, the disease is hidden, multi-system tired, clinical attention should be paid to screening secondary factors, especially the need to screen whether to combine systemic vasculitis, connective tissue diseases and other autoimmune diseases or malignant tumor-related diseases, enhance THE MRI examination of the diagnosis of the disease has a guiding significance, diagnosis depends on epidural biopsy Treatment to hormone sorority or hormone combined immunosuppressant treatment, there are reports that intra-injection dexamethasone and methotrexate helps to reduce the partial inflammation of the epidural, in the hormone reduction process or for various reasons lead to patients to stop oral hormones, especially to be alert to chronic headache, epilepsy, mental abnormalities and other symptoms recurrence, if the disease recurrence, can be considered to give a large dose of shock hormone therapy, while combining the use of immunosuppressants if the above treatment, still can not achieve satisfactory clinical results, it is necessary to consider whether to be related to its existing different pathogenesis Some patients may need surgical intervention if imaging suggests that epidural hyperplasia is evident, and that there has been a physical pressure on the brain or pressure on the brain, which may be treated with glucocorticoids or immunosuppressants