Childs Nerv Syst DOI 10.1007/s00381-015-2680-z
CASE-BASED UPDATE
Hypertrophic pachymeningitis Tomasz Dziedzic & Jakub Wojciechowski & Arkadiusz Nowak & Andrzej Marchel
Received: 1 March 2015 / Accepted: 4 March 2015 # Springer-Verlag Berlin Heidelberg 2015
Abstract Background Hypertrophic pachymeningitis (HP) is a rare clinical entity characterized by diffuse or localized fibrous thickening of the dura mater. It is well known but rare especially in pediatric population disease of differing origins. The primary (idiopathic) form is diagnosed after excluding other possible etiologies. Similar results from magnetic resonance imaging (MRI) for patients with hypertrophic pachymeningitis and meningiomas may make the diagnosis confusing. Additionally, making a proper diagnosis without histological sampling can be difficult in some cases. Case description We present a case of an 18-year-old boy diagnosed with hypertrophic pachymeningitis in the area of the hypoglossal canal. The diagnosis was made after a 2month history of hypoglossal nerve palsy and dysphagia preceded by a middle ear infection. The patient was treated surgically with suspicion of meningioma, but no evidence of a tumor was found during the operation. The postoperative period was uneventful. At the latest check-up, MRI revealed regression of all previously observed pathological changes. Keywords Meningioma . Cranial nerve palsy . Dural thickening . Hypoglossal nerve paralysis . Middle ear infection . Pediatric
Introduction
in 1818. Fifty-five years later, Charcot and Joffroy (1873) published a clinical picture of the disease [1]. There are two forms of the disease; the primary (idiopathic) form is diagnosed after excluding other possible etiologies, and the secondary form, which is caused by infection, autoimmune disorders, sarcoidosis, or a neoplastic process. Clinical symptoms like headaches, cranial nerve palsies, or myelopathy are caused by thickening of the cranial and/or spinal dura mater. The radiological pattern of this disease may mimic meningioma. Therefore, strong suspicion of a pathology that will not require surgical treatment should be borne in mind.
Histological findings HP is defined as thickening of the cranial or/and spinal dura mater, with inflammation evidenced by magnetic resonance imaging (MRI) or histology [2]. The pathogenic mechanism causing dural thickening is not fully understood, but it is considered to be an autoimmune disorder [3]. Macroscopically, the dura mater is grossly thickened and a pinkish-gray color [1]. The thickness of the dura matter varies from 5 to 15 mm, [4] with the mean thickness being 6.9 mm [5]. In microscopic examinations, abnormal dura mater is composed of multiple layers of fibrous connective tissue [4]. Final confirmation of HP is based on a biopsy of the involved dura mater [6, 7]. In some cases, as in the case presented here, this may be a diagnosis of exclusion.
Hypertrophic pachymeningitis (HP) is a rare clinical entity characterized by diffuse or localized fibrous thickening of the dura mater. The first case was recorded by Abercrombie
Epidemiology
T. Dziedzic (*) : J. Wojciechowski : A. Nowak : A. Marchel Department of Neurosurgery, Medical University of Warsaw, Banacha 1a, 02-097 Warszawa, Poland e-mail: [email protected]
The largest number of cases (159 patients) was presented by Yonekawa in a nationwide survey done in Japan [2]. HP is a disease affecting mainly adult patients (mean age 58.3 years) with a prevalence of 0.949/100 000 in the Japanese population
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[2]. So far, only 5 cases have been described in a pediatric group [8–12] (Table 1).
Etiology The idiopathic form is the most common, followed by antineutrophil cytoplasmic antibody (ANCA)-related HP, Bother infections^, IgG4-related HP, and other rare causes [2]. ANCA-related HP is often associated with such ANCArelated vascular diseases as microscopic polyangiitis, ChurgStrauss syndrome, Takayasu disease [13], or Wegner’s disease [14]. Patients with these diseases, who experience chronic headaches and associated multiple cranial nerve deficits, should be suspected of having HP. Other infections include infections caused by Taenia solium (neurocysticercosis) [15], Treponema pallidum (syphilis) [16], Epstein-Barr virus [17], fungi [18, 19], tuberculosis [20, 21], and middle ear infections [22]. Acute otitis media like that experienced by our case is most often initially caused by a viral infection that is followed by a secondary bacterial infection, usually with Streptococcus pneumoniae, Haemophilus influenza, or Moraxella catarrhalis [23]. IgG4-related HP is the intracranial form of fibrosing inflammatory disease that was initially diagnosed in patients with autoimmune pancreatitis in 2001 [24]. Since then, many cases of HP previously identified as idiopathic have been found to have high levels of IgG4 [25–32]. This type is confirmed by increased serum levels of IgG4 and by a finding of more than 10 IgG4 cells in a high power field [33, 34] during the histopathological examination. In some cases of HP, the levels of both ANCA and IgG4 can be higher [35]. Other rare causes of HP include long-term use of medications [36, 37] or autoimmune diseases [38, 39].
Clinical presentation Signs of cranial HP are related to the involvement of vascular or/and neural structures. Signs depend on the disease location Table 1
in the intracranial space [21, 40–43]. The most common signs include headaches and multiple cranial nerve dysfunctions [44]. Headache pain in HP can be divided into diffuse or focal types. Dura mater fibrosis can cause cerebral venous sinus occlusion and sinus thrombosis, which is usually associated with diffuse headaches [17, 45–53]. Decreased venous outflow after sinus occlusion results in decreased cerebrospinal fluid (CSF) resorption, which may cause hydrocephalus as well as global headaches [9]. Increased intracranial pressure related to decreased CSF resorption may also be responsible for basal encephalocele, which was the initial symptom of HP [54]. Another potential result of sinus occlusion in patients is formation of a dural arteriovenous fistula [55]. Unilateral headaches with ophthalmoplegia are characteristics of Tolosa-Hunt syndrome and involvement of the cavernous sinus [56–58]. Multiple cranial nerve palsies are the second most common sign of the disease [11, 59]. Monocular or bilateral visual loss can be related to dural thickening [60–64]. Thickening of the dura mater in the internal auditory canals may also cause hearing loss resulting from steroid therapy [65–67]. In rare cases, this disease may involve brain parenchyma and can cause epileptic seizure [68–70], brain edema with associated neurological deficit [71, 72], or psychiatric disorders [73]. Hypopituitarism secondary to pituitary insufficiency may also be an initial symptom of HP [74–76]. A spinal location of HP is more common than an intracranial one. According to the first description, the disease usually involves the dura mater of the posterior part of the cervical and thoracic spinal column [1, 16, 77–85]. The disease has been described in the cervical, thoracic, and lumbar spine. Sometimes, extensions from the spinal column into the posterior fossa may also occur [21, 40, 41]. A clinical picture of spinal HP, as in the initial description of the disease, is divided into three stages. In the first stage, the patient feels radicular pain followed by muscular weakness and spastic paralysis [4]. In 1925, Elsberg stated that HP must be suspected when a patient with signs of spinal cord compression has radicular pain referring to three or more nerve roots [4, 86–89].
Comparison of 5 reported cases of HP in children in chronological order
Age/gender
Symptoms
Location
Etiology
Therapy
Year
13 y/f 10 y/m 3,5 y/m 4 y/m 2 y/m
Headache, vomiting Diplopia Vomiting, irritability Symptoms of meningitis Visual loss, ptosis, seizures
Tentorial region Parasellar Cranial and spinal meninges Spinal Parasellar
Aplastic anemia Post-infectious Idiopathic Tuberculosis Tuberculosis
Medical Medical Medical Medical Medical
1999 [10] 2007 [11] 2009 [9] 2012 [12] 2014 [8]
Y years, f female, m male
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Radiographic examination Typical MRI findings in HP are thickening of the dura mater, with a low signal in T1 and T2 sequences with contrast enhancement. BPeace sign^ [90] and BEiffel by night sign^ [91] are characteristics of HP. Imaging usually does not reveal any associated osteolytic [79], osteoblastic [92], or superficial soft tissue masses [93]. In most cases, those lesions are extra-axial, but cases with brain parenchyma involvement [70] with accompanied brain edema [71, 72] have also been reported. Those changes are often multifocal and may mimic multiple meningiomas [94, 95], neurosarcoidosis [96, 97], lymphoma [98], or acute subdural hematoma [99]. Due to difficulties in radiological diagnosis, meningioma [100] and neuroblastoma
Fig. 1 a–c Initial magnetic resonance imaging. White arrow indicates a tumor located in the hypoglossal canal and posterior fossa. d–f Preoperative imaging with observed tumor enlargement compared to
[101] were taken for pachymeningitis. Positron emission tomography can be helpful as an additional tool in doubtful cases [102]. In the case of spinal HP, MRI is also a diagnostic modality of choice. In the past, a lateral myelogram was performed and the spinal cord was pushed anteriorly. Additionally, total block in the lumbar puncture was found and CSF sampling revealed albumin-cytological dissociation [4].
Treatment Even though spontaneous resolution of the disease is likely to occur, most symptomatic patients require treatment [103]. In
the initial examination. a, d Axial T1-weighted imaging. b, e Postcontrast axial T1-weighted imaging. c, f Axial T2-weighted imaging
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most cases of idiopathic HP, a good initial response to steroids is observed. In some cases, steroids combined with immunosuppressive medications may be necessary [44]. In other types of HP, targeted therapy is recommended and is usually combined with steroids. Patients who are unresponsive to medical therapy and have progressive neurological deficits are candidates for surgical therapy. The first attempts at surgical treatment of spinal HP were made in 1901 by Schede [1]. Surgical techniques have varied from decompressive laminectomies and incisions of the dura mater to its partial extirpation [1]. Surgical treatment can be very difficult due to the possibility of dense adhesions to the spinal cord [1].
Prognosis Long-term follow-up results are favorable, and there is usually no recurrence [47, 104, 105]. Relapse or recurrence [105–108] is associated with termination of medical therapy too soon, including steroid therapy [44].
Exemplary case History Our patient was an 18-year-old boy with a history of left hypoglossal nerve paralysis, hoarseness, and paresis of the soft palate on the left side. Two months before admission, the patient was diagnosed with otitis media in the left ear. One month later, the patient complained of painful edema on the left side of his tongue. Those symptoms were followed by dysphagia and hoarseness. During performance of an indirect laryngoscopy, paresis of the left vocal cord was visualized. Antibiotics were prescribed for 2 weeks because of the suspected infectious origin of the symptoms. Examination On admission, the patient had left hypoglossal nerve paralysis, hoarseness, and paresis of the soft palate on the left side. There were no deficits in the upper and lower extremities. In the initial magnetic resonance imaging (MRI), the lesion appeared to be hypointense in the T1 and T2 sequences, with intense and homogenous contrast enhancement. The tumor was located mainly in the posterior fossa with extension to the hypoglossal canal. In a MRI performed 1 month later, enlargement of the tumor was observed (Fig. 1). With computed tomography (CT), no widening of the hypoglossal canal on the left side compared to the right side was observed (Fig. 2). Osteoblastic or osteoclastic changes around the canal were also not observed.
Fig. 2 Preoperative computed tomography scan showing the equal size of both hypoglossal canals
Operation A far lateral approach with posterior, partial condylectomy was chosen to remove the tumor. Dense arachnoid adhesions of the cerebellar hemisphere with the normal appearing dura mater below the jugular foramen were the only intraoperative findings. No histological samples were taken due to the lack of any visible changes. The vertebral artery, hypoglossal nerve, lower cranial nerves (IX, X, XI, XII), and the facial and vestibulocochlear nerves also showed no changes. Postoperative course The patient’s postoperative course was uneventful. One month after surgery, the MRI appeared normal with no abnormal changes (Fig. 1), although the neurological deficits that were seen on admission did not resolve during long-term follow-up. Based on the clinical course and intraoperative findings, secondary HP after middle ear infection was diagnosed. Additional testing for other possible causes of secondary HP was not performed.
Summary and management recommendation HP is rare as an intracranial disease in children and is radiologically similar to meningiomas. The presented case demonstrates that in some cases, it can be very similar in radiological appearance. Final confirmation of HP is based on a biopsy of the involved dura mater. In some cases, as in the case presented here, this may be a diagnosis of exclusion. Even though spontaneous resolution of the disease is likely to occur, most of symptomatic patients require treatment. Medical treatment is the first choice in HP.
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Patients who are unresponsive to medical therapy and have progressive neurological deficits are candidates for surgical therapy. HP must be considered as a differential diagnosis in skull base lesions with rapid progression of radiological findings and clinical signs.
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92. Lin CK, Lai DM (2013) IgG4-related intracranial hypertrophic pachymeningitis with skull hyperostosis: a case report. BMC Surg 13:37 93. Keshavaraj A, Gamage R, Jayaweera G, Gooneratne IK (2012) Idiopathic hypertrophic pachymeningitis presenting with a superficial soft tissue mass. J Neurosci Rural Pract 3:193–195 94. Hosler MR, Turbin RE, Cho ES, Wolansky LJ, Frohman LP (2007) I d io p at h i c h y p e r tr o p h i c p a c h y m e n i n g i t i s m i m i ck i n g lymphoplasmacyte-rich meningioma. J Neuroophthalmol Off J N Am Neuroophthalmol Soc 27:95–98 95. Deprez M, Born J, Hauwaert C, Otto B, Reznik M (1997) Idiopathic hypertrophic cranial pachymeningitis mimicking multiple meningiomas: case report and review of the literature. Acta Neuropathol 94: 385–389 96. Roche JC, Sanchez-Carteyron A, Minchole E, Ara JR, Alarcia R (2012) Reply: idiopathic hypertrophic pachymeningitis mimicking neurosarcoidosis. Clin Neurol Neurosurg 114:825–826 97. Christakis PG, Machado DG, Fattahi P (2012) Idiopathic hypertrophic pachymeningitis mimicking neurosarcoidosis. Clin Neurol Neurosurg 114:176–178 98. Ishiyama K, Okada M, Anzai N, Tashima M (2011) Mantle cell lymphoma manifesting neurological symptoms similar to those of hypertrophic cranial pachymeningitis. [Rinsho ketsueki] Jpn J Clin Hematol 52:1788–1793 99. Park IS, Kim H, Chung EY, Cho KW (2010) Idiopathic hypertrophic cranial pachymeningitis misdiagnosed as acute subtentorial hematoma. J Korean Neurosurg Soc 48:181–184 100. Hirunwiwatkul P, Trobe JD, Blaivas M (2007) Lymphoplasmacyterich mening ioma mimicking id iopathi c hy pertrophic pachymeningitis. J Neuroophthalmol Off J N Am Neuroophthalmol Soc 27:91–94 101. Singh P, Verma R, Paliwal VK, Neyaz Z (2012) Neuroblastoma presenting as hypertrophic pachymeningitis in a young female. Neurol India 60:655–656 102. Norikane T, Yamamoto Y, Okada M, Maeda Y, Aga F, Kawai N, Nishiyama Y (2012) Hypertrophic cranial pachymeningitis with IgG4-positive plasma cells detected by C-11 methionine PET. Clin Nucl Med 37:108–109 103. Nishio S, Morioka T, Togawa A, Yanase T, Nawata H, Fukui M, Hasuo K (1995) Spontaneous resolution of hypertrophic cranial pachymeningitis. Neurosurg Rev 18:201–204 104. Karakasis C, Deretzi G, Rudolf J, Tsiptsios I (2012) Long-term lack of progression after initial treatment of idiopathic hypertrophic pachymeningitis. J Clin Neurosci Off J Neurosurg Soc Australas 19:321–323 105. Tsutsui M, Yasuda T, Kanamori M, Hori T, Kimura T (2012) Longterm outcome of idiopathic hypertrophic thoracic pachymeningitis. Eur Spine J Off Publ Eur Spine Soc Eur Spinal Deform Soc Eur Sect Cerv Spine Res Soc 21(Suppl 4):S404–S407 106. Denays R, Milbouw G, Mosseray A, Michotte A, Hella P (2013) Focal idiopathic hypertrophic pachymeningitis: a case with two separate sites involved at a 5-year interval. Acta Neurol Belg 113: 95–98 107. Ito Z, Osawa Y, Matsuyama Y, Aoki T, Harada A, Ishiguro N (2006) Recurrence of hypertrophic spinal pachymeningitis. Report of two cases and review of the literature. J Neurosurg Spine 4:509–513 108. Claus E, Rutgers M, Sindic C, Raftopoulos C, Godfraind C, Duprez T (2005) Remitting/relapsing idiopathic hypertrophic spinal pachymeningitidis: comprehensive imaging work-up and MR monitoring. Eur Radiol 15:53–58
CASE-BASED UPDATE
Hypertrophic pachymeningitis Tomasz Dziedzic & Jakub Wojciechowski & Arkadiusz Nowak & Andrzej Marchel
Received: 1 March 2015 / Accepted: 4 March 2015 # Springer-Verlag Berlin Heidelberg 2015
Abstract Background Hypertrophic pachymeningitis (HP) is a rare clinical entity characterized by diffuse or localized fibrous thickening of the dura mater. It is well known but rare especially in pediatric population disease of differing origins. The primary (idiopathic) form is diagnosed after excluding other possible etiologies. Similar results from magnetic resonance imaging (MRI) for patients with hypertrophic pachymeningitis and meningiomas may make the diagnosis confusing. Additionally, making a proper diagnosis without histological sampling can be difficult in some cases. Case description We present a case of an 18-year-old boy diagnosed with hypertrophic pachymeningitis in the area of the hypoglossal canal. The diagnosis was made after a 2month history of hypoglossal nerve palsy and dysphagia preceded by a middle ear infection. The patient was treated surgically with suspicion of meningioma, but no evidence of a tumor was found during the operation. The postoperative period was uneventful. At the latest check-up, MRI revealed regression of all previously observed pathological changes. Keywords Meningioma . Cranial nerve palsy . Dural thickening . Hypoglossal nerve paralysis . Middle ear infection . Pediatric
Introduction
in 1818. Fifty-five years later, Charcot and Joffroy (1873) published a clinical picture of the disease [1]. There are two forms of the disease; the primary (idiopathic) form is diagnosed after excluding other possible etiologies, and the secondary form, which is caused by infection, autoimmune disorders, sarcoidosis, or a neoplastic process. Clinical symptoms like headaches, cranial nerve palsies, or myelopathy are caused by thickening of the cranial and/or spinal dura mater. The radiological pattern of this disease may mimic meningioma. Therefore, strong suspicion of a pathology that will not require surgical treatment should be borne in mind.
Histological findings HP is defined as thickening of the cranial or/and spinal dura mater, with inflammation evidenced by magnetic resonance imaging (MRI) or histology [2]. The pathogenic mechanism causing dural thickening is not fully understood, but it is considered to be an autoimmune disorder [3]. Macroscopically, the dura mater is grossly thickened and a pinkish-gray color [1]. The thickness of the dura matter varies from 5 to 15 mm, [4] with the mean thickness being 6.9 mm [5]. In microscopic examinations, abnormal dura mater is composed of multiple layers of fibrous connective tissue [4]. Final confirmation of HP is based on a biopsy of the involved dura mater [6, 7]. In some cases, as in the case presented here, this may be a diagnosis of exclusion.
Hypertrophic pachymeningitis (HP) is a rare clinical entity characterized by diffuse or localized fibrous thickening of the dura mater. The first case was recorded by Abercrombie
Epidemiology
T. Dziedzic (*) : J. Wojciechowski : A. Nowak : A. Marchel Department of Neurosurgery, Medical University of Warsaw, Banacha 1a, 02-097 Warszawa, Poland e-mail: [email protected]
The largest number of cases (159 patients) was presented by Yonekawa in a nationwide survey done in Japan [2]. HP is a disease affecting mainly adult patients (mean age 58.3 years) with a prevalence of 0.949/100 000 in the Japanese population
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[2]. So far, only 5 cases have been described in a pediatric group [8–12] (Table 1).
Etiology The idiopathic form is the most common, followed by antineutrophil cytoplasmic antibody (ANCA)-related HP, Bother infections^, IgG4-related HP, and other rare causes [2]. ANCA-related HP is often associated with such ANCArelated vascular diseases as microscopic polyangiitis, ChurgStrauss syndrome, Takayasu disease [13], or Wegner’s disease [14]. Patients with these diseases, who experience chronic headaches and associated multiple cranial nerve deficits, should be suspected of having HP. Other infections include infections caused by Taenia solium (neurocysticercosis) [15], Treponema pallidum (syphilis) [16], Epstein-Barr virus [17], fungi [18, 19], tuberculosis [20, 21], and middle ear infections [22]. Acute otitis media like that experienced by our case is most often initially caused by a viral infection that is followed by a secondary bacterial infection, usually with Streptococcus pneumoniae, Haemophilus influenza, or Moraxella catarrhalis [23]. IgG4-related HP is the intracranial form of fibrosing inflammatory disease that was initially diagnosed in patients with autoimmune pancreatitis in 2001 [24]. Since then, many cases of HP previously identified as idiopathic have been found to have high levels of IgG4 [25–32]. This type is confirmed by increased serum levels of IgG4 and by a finding of more than 10 IgG4 cells in a high power field [33, 34] during the histopathological examination. In some cases of HP, the levels of both ANCA and IgG4 can be higher [35]. Other rare causes of HP include long-term use of medications [36, 37] or autoimmune diseases [38, 39].
Clinical presentation Signs of cranial HP are related to the involvement of vascular or/and neural structures. Signs depend on the disease location Table 1
in the intracranial space [21, 40–43]. The most common signs include headaches and multiple cranial nerve dysfunctions [44]. Headache pain in HP can be divided into diffuse or focal types. Dura mater fibrosis can cause cerebral venous sinus occlusion and sinus thrombosis, which is usually associated with diffuse headaches [17, 45–53]. Decreased venous outflow after sinus occlusion results in decreased cerebrospinal fluid (CSF) resorption, which may cause hydrocephalus as well as global headaches [9]. Increased intracranial pressure related to decreased CSF resorption may also be responsible for basal encephalocele, which was the initial symptom of HP [54]. Another potential result of sinus occlusion in patients is formation of a dural arteriovenous fistula [55]. Unilateral headaches with ophthalmoplegia are characteristics of Tolosa-Hunt syndrome and involvement of the cavernous sinus [56–58]. Multiple cranial nerve palsies are the second most common sign of the disease [11, 59]. Monocular or bilateral visual loss can be related to dural thickening [60–64]. Thickening of the dura mater in the internal auditory canals may also cause hearing loss resulting from steroid therapy [65–67]. In rare cases, this disease may involve brain parenchyma and can cause epileptic seizure [68–70], brain edema with associated neurological deficit [71, 72], or psychiatric disorders [73]. Hypopituitarism secondary to pituitary insufficiency may also be an initial symptom of HP [74–76]. A spinal location of HP is more common than an intracranial one. According to the first description, the disease usually involves the dura mater of the posterior part of the cervical and thoracic spinal column [1, 16, 77–85]. The disease has been described in the cervical, thoracic, and lumbar spine. Sometimes, extensions from the spinal column into the posterior fossa may also occur [21, 40, 41]. A clinical picture of spinal HP, as in the initial description of the disease, is divided into three stages. In the first stage, the patient feels radicular pain followed by muscular weakness and spastic paralysis [4]. In 1925, Elsberg stated that HP must be suspected when a patient with signs of spinal cord compression has radicular pain referring to three or more nerve roots [4, 86–89].
Comparison of 5 reported cases of HP in children in chronological order
Age/gender
Symptoms
Location
Etiology
Therapy
Year
13 y/f 10 y/m 3,5 y/m 4 y/m 2 y/m
Headache, vomiting Diplopia Vomiting, irritability Symptoms of meningitis Visual loss, ptosis, seizures
Tentorial region Parasellar Cranial and spinal meninges Spinal Parasellar
Aplastic anemia Post-infectious Idiopathic Tuberculosis Tuberculosis
Medical Medical Medical Medical Medical
1999 [10] 2007 [11] 2009 [9] 2012 [12] 2014 [8]
Y years, f female, m male
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Radiographic examination Typical MRI findings in HP are thickening of the dura mater, with a low signal in T1 and T2 sequences with contrast enhancement. BPeace sign^ [90] and BEiffel by night sign^ [91] are characteristics of HP. Imaging usually does not reveal any associated osteolytic [79], osteoblastic [92], or superficial soft tissue masses [93]. In most cases, those lesions are extra-axial, but cases with brain parenchyma involvement [70] with accompanied brain edema [71, 72] have also been reported. Those changes are often multifocal and may mimic multiple meningiomas [94, 95], neurosarcoidosis [96, 97], lymphoma [98], or acute subdural hematoma [99]. Due to difficulties in radiological diagnosis, meningioma [100] and neuroblastoma
Fig. 1 a–c Initial magnetic resonance imaging. White arrow indicates a tumor located in the hypoglossal canal and posterior fossa. d–f Preoperative imaging with observed tumor enlargement compared to
[101] were taken for pachymeningitis. Positron emission tomography can be helpful as an additional tool in doubtful cases [102]. In the case of spinal HP, MRI is also a diagnostic modality of choice. In the past, a lateral myelogram was performed and the spinal cord was pushed anteriorly. Additionally, total block in the lumbar puncture was found and CSF sampling revealed albumin-cytological dissociation [4].
Treatment Even though spontaneous resolution of the disease is likely to occur, most symptomatic patients require treatment [103]. In
the initial examination. a, d Axial T1-weighted imaging. b, e Postcontrast axial T1-weighted imaging. c, f Axial T2-weighted imaging
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most cases of idiopathic HP, a good initial response to steroids is observed. In some cases, steroids combined with immunosuppressive medications may be necessary [44]. In other types of HP, targeted therapy is recommended and is usually combined with steroids. Patients who are unresponsive to medical therapy and have progressive neurological deficits are candidates for surgical therapy. The first attempts at surgical treatment of spinal HP were made in 1901 by Schede [1]. Surgical techniques have varied from decompressive laminectomies and incisions of the dura mater to its partial extirpation [1]. Surgical treatment can be very difficult due to the possibility of dense adhesions to the spinal cord [1].
Prognosis Long-term follow-up results are favorable, and there is usually no recurrence [47, 104, 105]. Relapse or recurrence [105–108] is associated with termination of medical therapy too soon, including steroid therapy [44].
Exemplary case History Our patient was an 18-year-old boy with a history of left hypoglossal nerve paralysis, hoarseness, and paresis of the soft palate on the left side. Two months before admission, the patient was diagnosed with otitis media in the left ear. One month later, the patient complained of painful edema on the left side of his tongue. Those symptoms were followed by dysphagia and hoarseness. During performance of an indirect laryngoscopy, paresis of the left vocal cord was visualized. Antibiotics were prescribed for 2 weeks because of the suspected infectious origin of the symptoms. Examination On admission, the patient had left hypoglossal nerve paralysis, hoarseness, and paresis of the soft palate on the left side. There were no deficits in the upper and lower extremities. In the initial magnetic resonance imaging (MRI), the lesion appeared to be hypointense in the T1 and T2 sequences, with intense and homogenous contrast enhancement. The tumor was located mainly in the posterior fossa with extension to the hypoglossal canal. In a MRI performed 1 month later, enlargement of the tumor was observed (Fig. 1). With computed tomography (CT), no widening of the hypoglossal canal on the left side compared to the right side was observed (Fig. 2). Osteoblastic or osteoclastic changes around the canal were also not observed.
Fig. 2 Preoperative computed tomography scan showing the equal size of both hypoglossal canals
Operation A far lateral approach with posterior, partial condylectomy was chosen to remove the tumor. Dense arachnoid adhesions of the cerebellar hemisphere with the normal appearing dura mater below the jugular foramen were the only intraoperative findings. No histological samples were taken due to the lack of any visible changes. The vertebral artery, hypoglossal nerve, lower cranial nerves (IX, X, XI, XII), and the facial and vestibulocochlear nerves also showed no changes. Postoperative course The patient’s postoperative course was uneventful. One month after surgery, the MRI appeared normal with no abnormal changes (Fig. 1), although the neurological deficits that were seen on admission did not resolve during long-term follow-up. Based on the clinical course and intraoperative findings, secondary HP after middle ear infection was diagnosed. Additional testing for other possible causes of secondary HP was not performed.
Summary and management recommendation HP is rare as an intracranial disease in children and is radiologically similar to meningiomas. The presented case demonstrates that in some cases, it can be very similar in radiological appearance. Final confirmation of HP is based on a biopsy of the involved dura mater. In some cases, as in the case presented here, this may be a diagnosis of exclusion. Even though spontaneous resolution of the disease is likely to occur, most of symptomatic patients require treatment. Medical treatment is the first choice in HP.
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Patients who are unresponsive to medical therapy and have progressive neurological deficits are candidates for surgical therapy. HP must be considered as a differential diagnosis in skull base lesions with rapid progression of radiological findings and clinical signs.
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