[One case per day] Chinese Journal of Neurosurgery: A case of primary central nervous system vasculitis with magnetic resonance-like brain tumors

Fig.

Fig.

Primary angiitis of the central nervous system (PACNS) is a rare autoimmune disease

Summary of medical records

The patient is a male, 63 years old

Physical examination: right lower extremity muscle strength IV.

After admission, the diagnosis is a left parietal lobe mass lesion, and the glioma may be large

Cranial MRI (Figure 3b) shows a left parietal lobe clumpy confounding signal, and cranial CTA (Figure 3c) does not see intracranial artery stenosis and dilation

Initially, methylprednisolone 1 g/day×3 shock therapy is used, and the patient's symptoms are still progressively worsening

First case discussion (after surgery)

The patient is an elderly man, the course of the disease is short, clinically manifested as symptoms of impaired nerve function, physical examination suggests damage to the left upper motor neuron, previous history of hypertension, MRI sees left parietal clump-like mixed signal shadow, mainly long T1 long ta signal, irregular reinforcement, surrounding edema band, preoperative diagnosis of glioma is likely, surgical treatment

The second case discussion (after recurrence) The patient's postoperative lesions were sent to the pathology department with several pieces of gray powder and gray-brown tissue, and the lesions were mainly large pieces of necrotic tissue under the conventional paraffin fixed embedding H&E staining microscope, no tumor cells were seen, a large number of lymphocytes and a small amount of neutrophil infiltration could be seen around the blood vessels, and GFAP (+), CD68 (+), NeuN (+), CD3 (+), CD20 (partial +)

Beijing Institute of Neurosurgery consulting pathological consultation slices can be seen: (1) irregular perivascular and vascular wall inflammatory cells, lymphocytes mainly

Consultation opinions of the Department of Pathology of Beijing Xuanwu Hospital: coagulated necrosis in brain tissue, degeneration and necrosis of blood vessel walls, endovascular hyperplasia, perivascular lymphocyte infiltration, piaetrachritis cell infiltration, special staining PAS without fungus, acid-resistance staining (-), consider vasculitis with large necrosis

2 months after the operation, the patient's symptoms recurred, and the review of MRI saw that the left parietal lobe reappeared as a clumpy mixed signal shadow, mainly the long T1 long ta signal, the performance was roughly the same, combined with the pathological consultation results of different hospitals, the vascular wall lymphocyte infiltration was mainly, and vasculitis was not excluded, in summary, considering the possibility of vasculitic lesions in the left parietal lobe of the patient, there was insufficient evidence to support brain tumors

Pathology suggests inflammatory cell infiltration of vascular walls, mainly lymphocytes, which is more specific, and is different from vascular inflammation secondary to tumors and infections, which is mainly manifested as infiltration of inflammatory cells around blood vessels

Third case discussion (2 years after immunosuppressive therapy)

After long-term glucocorticoid and cyclophosphamide therapy, the clinical manifestations gradually remissioned and the imaging lesions gradually decreased, which can confirm the diagnosis of

PACNS is clinically rare, but about 5% of PACNS presents as a mass-like lesion, with similar clinical manifestations and imaging to brain tumors, cranial CTA, cerebrospinal fluid examination, inflammatory indicators, immunological antibodies and other examinations can assist in differentiation, and brain tissue biopsy helps to confirm the diagnosis

The characteristics of PACNS are summarized below: PACNS is a rare, highly heterogeneous immune system disease, manifested as multiple and segmental small and medium-vessel inflammation of the central nervous system, not accompanied by evidence
of systemic vasculitis or secondary vasculitis.
Clinically, PACNS can be divided into two categories: (1) granulomatous vasculitis (GACN) of the central nervous system: pathological manifestations are typical of granulomatous vasculitis
.
(2) Atypical PACNS: one of the following conditions is met (1) pathological manifestations are lymphocytic vasculitis, (2) diagnosis by cerebral angiography or MRI without pathological biopsy
.

The disease can occur in people of all ages, with an average age of about 42 years at diagnosis; It is more common in men, and it has been reported that the ratio of men to women is 2:1
.
PACNS has an insidious onset and develops slowly
.
Since vasculitis can affect different areas of the central nervous system, the clinical manifestations are diverse
.
In a study of 116 patients, 70% of patients developed neurologic deficits, with cognitive changes (83%), headache (56%), epilepsy (30%), stroke (14%), and intracerebral hemorrhage (12%)
more common.
Spinal cord involvement may also present.

Clinically, in patients with suspected PACNS, the following tests are helpful in diagnosis, but there are no specific tests at present: (1) laboratory tests: acute phase erythrocyte sedimentation rate, C-reactive protein is normal
.
If elevated, infection, systemic vasculitis, etc.
should be considered
.
(2) Serological examination: the main purpose is to exclude possible connective tissue diseases
.
Tests that need to be done include: antinuclear antibodies, rheumatoid factor, anti-Ro/SSA, La/SSB, Sm, RNP antibodies, anti-ds-DNA antibodies, anti-neutrophil cytoplasm antibodies, complement, immunoglobulin quantification, and electrophoresis
.
(3) Cerebrospinal fluid examination: 80% of PATIENTS with CEREBROS HAVE ABNORMAL CEREBROSAL FLUID, such as: moderate lymphocytosis, increased protein content, normal glucose content, and negative bacterial fungal culture
.

(4) Neuroimaging manifestations: MRI examination is manifested as single or multiple occurrences of brain tissue necrosis, bleeding or tumor-like changes, typical manifestations are mainly multiple, bilateral cerebral cortex, deep white matter, meningeal lesions, with or without intensification
.
5% of patients may present with clump-like lesions
.
Local microcirculation abnormalities are seen in perfusion-weighted imaging (PWI) and small, active ischemic foci
are seen on diffusion-weighted imaging (DWI).
(5) Cerebral angiography: typically manifested as alternating vascular stenosis and dilation, mainly involving arterioles, and multiple lesions help to confirm the diagnosis
of PACNS.
Since PACNS mainly involves small blood vessels, the sensitivity of cerebral angiography is not high, about 60%.

A positive cerebral angiography is helpful in the diagnosis of vasculitis, but it is impossible to exclude vasculitis caused by other causes, so the diagnostic specificity of PACNS is not high
.

(6) Brain tissue biopsy: Brain tissue biopsy is the gold standard for PACNS diagnosis
.
Due to the limitations of the site of the material, the sensitivity of the biopsy is about 75%.

Pathological manifestations are: (1) granulomatous: about 50%, manifested by mononucleosis and granuloma formation, langerhans cells
can be seen.
(2) Atypical PACNS: pathological granulomas can be called atypical PACNS, and common manifestations include paravascular and wall lymphocyte infiltration
.
Bacterial, fungal culture, viral testing, and immunohistochemical staining of biopsy tissue can help rule out similar lesions
.

Current treatments are not supported by clinical trials
.
If a pathological diagnosis is already made, immunosuppressive therapy
should be initiated.
Patients with GACNS should be treated
with a combination of glucocorticoids and cyclophosphamide.
For patients with atypical PACNS, the treatment regimen should be individualized, the initial dose of hormones should be sufficient, the dose should be slow, and the possibility
of infectious disease should be comprehensively investigated before treatment.
(1) Glucocorticoids: high-dose hormones are used for early control
of the disease.
The starting dose is prednisone 1 mg/kg·d, up to 80 mg/day; Methylprednisolone 15 mg/kg·d shock therapy can also be used for 3 days, and the conventional dose
is switched to on day 4.

It has been reported in the literature that those with poor effect of methotrexone impact can be injected intrathecally
with methylprednisolone + methotrexate.
(2) Cyclophosphamide: daily oral medication or intermittent intravenous medication regimen can be used
.
When administered daily orally, the daily dose is 1.
5 to 2.
0 mg/kg·d, and the level of white blood cells is measured during the administration
.
(3) Efficacy evaluation: The evaluation method of treatment effect includes subjective symptoms, signs and imaging examination
of patients.
Due to the presence of infarction foci, some of the patient's symptoms do not fully recover
.
The absence of new lesions on imaging is an important manifestation of
disease remission.
(4) Course of treatment and recurrence: There is currently no data to suggest the appropriate medication time and treatment after the recurrence of the disease, and the clinical experience of clinicians in the treatment of other types of vasculitis can be followed
.