J Med Cases

Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc

Journal website https://www.journalmc.org

Case Report

Volume 14, Number 12, December 2023, pages 405-412

Idiopathic Hypertrophic Spinal Pachymeningitis

Figure 1. MRI of the brain. (a) T1-weighted MRI of the brain sagittal section post-contrast that reveals dural thickening of the posterior fossa indicated by the red arrow. (b) T1-weighted MRI of the brain axial section post-contrast that reveals a right cerebellopontine angle mass revealed by the blue arrow and dural thickening of the posterior fossa indicated by the red arrow. (c) T2-weighted MRI of the brain axial section that reveals a right cerebellopontine angle mass revealed by the blue arrow. MRI: magnetic resonance imaging.

Figure 2. MRI cervical spine. (a, b) T1-weighted MRI spine sagittal section without and with contrast showing cervical spine dural thickening with narrowing of cord respectively. (c, d) T1-weighted MRI spine without and with contrast axial section showing narrowing of cord respectively. MRI: magnetic resonance imaging.

Figure 3. MRI cervical spine. Follow-up T1-weighted MRI with contrast showing decreased meningeal enhancement and narrowing. (a) Sagittal view. (b) Axial view. MRI: magnetic resonance imaging.

**Table 1.** CSF Analysis
	2020	2015	2014	2013	Reference range
^aAbnormal results. Significant increase in CSF protein at the time the patient presented with neurologic symptoms is shown. AFB: acid-fast bacilli; CSF: cerebrospinal fluid.
Protein (mg/dL)	1,620^a	263^a	243^a	257^a	15 - 45
Glucose (mmol/L)	49	52	54	54	40 - 85
White cell count (cells/µL)	188^a, lymphocyte predominance	46^a, lymphocyte predominance	Not available	Not available	0 - 5
Red blood cell count (cells/µL)	103^a	Not available	Not available	Not available	0 - 10
AFB culture	Negative	Not available	Not available	Not available	Negative

Table 1. CSF Analysis

2020

2015

2014

2013

Reference range

^aAbnormal results. Significant increase in CSF protein at the time the patient presented with neurologic symptoms is shown. AFB: acid-fast bacilli; CSF: cerebrospinal fluid.

Protein (mg/dL)

1,620^a

263^a

243^a

257^a

15 - 45

Glucose (mmol/L)

40 - 85

White cell count (cells/µL)

188^a, lymphocyte predominance

46^a, lymphocyte predominance

Not available

0 - 5

Red blood cell count (cells/µL)

103^a

Not available

0 - 10

AFB culture

Negative

Not available

Negative

**Table 2.** Causes of Hypertrophic Pachymeningitis
ANCA: antineutrophil cytoplasmic antibody.
Inflammatory/autoimmune [23-25]	Idiopathic (67%) ANCA-associated vasculitis (15%) IgG4-related disease Sarcoidosis Rheumatoid arthritis Sjogren syndrome Systemic lupus erythematosus Giant-cell arteritis
Infectious [24]	Tuberculous meningitis (8%) Viral meningitis (6%) Bacterial meningitis (4%)
Neoplastic [24]	Lymphoma Meningioma Histiocytosis Carcinomatous meningitis
Other [24]	Previous surgical procedure Cerebrospinal fluid hypotension

Table 2. Causes of Hypertrophic Pachymeningitis

ANCA: antineutrophil cytoplasmic antibody.

Inflammatory/autoimmune [23-25]

Idiopathic (67%)
ANCA-associated vasculitis (15%)
IgG4-related disease
Sarcoidosis
Rheumatoid arthritis
Sjogren syndrome
Systemic lupus erythematosus
Giant-cell arteritis

Infectious [24]

Tuberculous meningitis (8%)
Viral meningitis (6%)
Bacterial meningitis (4%)

Neoplastic [24]

Lymphoma
Meningioma
Histiocytosis
Carcinomatous meningitis

Other [24]

Previous surgical procedure
Cerebrospinal fluid hypotension

**Table 3.** Outcomes of IHSP With Different Clinical Variables
Clinical variable	Improved	Partially improved	Not improved	Total
^aInitial intervention was considered in type of management. CRP: C-reactive protein; CSF: cerebrospinal fluid; ESR: erythrocytic sedimentation rate; IHSP: idiopathic hypertrophic spinal pachymeningitis; RA: rheumatoid arthritis; UMN: upper motor neuron.
Course of disease
Acute (≤ 24 h)	1 (25%)	1 (25%)	2 (50%)	4
Progressive (> 24 h)	10 (59%)	5 (29%)	2 (12%)	17
Clinical features
UMN signs	5 (50%)	1 (10%)	4 (40%)	10
CSF findings
Elevated proteins and leukocyte	4 (80%)	0	1 (20%)	5
Inflammatory markers
Elevated ESR, CRP and RA	3 (37.5%)	3 (37.5%)	2 (25%)	8
No. of spinal segments
≥ 6	5 (45.4%)	4 (36.3%)	2 (18.1%)	11
≤ 5	6 (60%)	2 (20%)	2 (20%)	10
Type of management^a
Surgical	3 (75%)	1 (25%)	0	4
Conservative	4 (100%)	0	0	4
Both surgical and conservative	4 (30.7%)	5 (38.5%)	4 (30.7%)	13
Location of IHSP
Anterior and posterior	1 (33.3%)	2 (66.6%)	0	3
Anterior	3 (75%)	0	1 (25%)	4
Posterior	2 (33.3%)	2 (33.3%)	2 (33.3%)	6
Circumferential	4 (57.1%)	2 (28.5%)	1 (14.2%)	7

Table 3. Outcomes of IHSP With Different Clinical Variables

Clinical variable

Improved

Partially improved

Not improved

Total

^aInitial intervention was considered in type of management. CRP: C-reactive protein; CSF: cerebrospinal fluid; ESR: erythrocytic sedimentation rate; IHSP: idiopathic hypertrophic spinal pachymeningitis; RA: rheumatoid arthritis; UMN: upper motor neuron.

Course of disease

Acute (≤ 24 h)

1 (25%)

2 (50%)

Progressive (> 24 h)

10 (59%)

5 (29%)

2 (12%)

Clinical features

UMN signs

5 (50%)

1 (10%)

4 (40%)

CSF findings

Elevated proteins and leukocyte

4 (80%)

1 (20%)

Inflammatory markers

Elevated ESR, CRP and RA

3 (37.5%)

2 (25%)

No. of spinal segments

≥ 6

5 (45.4%)

4 (36.3%)

2 (18.1%)

≤ 5

6 (60%)

2 (20%)

Type of management^a

Surgical

3 (75%)

1 (25%)

Conservative

4 (100%)

Both surgical and conservative

4 (30.7%)

5 (38.5%)

4 (30.7%)

Location of IHSP

Anterior and posterior

1 (33.3%)

2 (66.6%)

3 (75%)

1 (25%)

Posterior

2 (33.3%)

Circumferential

4 (57.1%)

2 (28.5%)

1 (14.2%)