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|LETTER TO EDITOR
|Year : 2020 | Volume
| Issue : 3 | Page : 706-707
Pachymeningitis in Patient with Lung Cancer: A Diagnostic Challenge
Enrico Ferrante, Giuseppe Pontrelli, Michele Trimboli
Department of Neurology, “A.O.R. San Carlo” Hospital, Potenza, Italy
|Date of Web Publication||6-Jul-2020|
Dr. Enrico Ferrante
A.O.R. San Carlo, Via Potito Petrone - 85100 Potenza
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Ferrante E, Pontrelli G, Trimboli M. Pachymeningitis in Patient with Lung Cancer: A Diagnostic Challenge. Neurol India 2020;68:706-7
Spontaneous intracranial hypotension (SIH) typically results from a spontaneous cerebrospinal fluid (CSF) leak at spine level, especially at the thoracic spine or cervicothoracic junction, which leads to a decrease in CSF volume. The exact cause of the spontaneous CSF leak often remains undetermined. Treatment ranges from conservative management, such as bed rest, over-hydration, and caffeine, to invasive procedures, such as the autologous epidural blood patch (EBP).
A 64-year-old male underwent left pneumectomy for squamous cell lung cancer (TNM staging: T2a N0 M0); the surgery was defined radical (R0) and histological grading was G3 (poorly differentiated). One year later, he presented with 1-month history of severe orthostatic headache following a strong coughing. Headache kept on worsening in following days changing from orthostatic to nonpositional and persistent one. Brain computed tomography (CT) scan in Emergency Room showed left frontal anterior convexity subarachnoid hemorrhage (SAH) [Figure 1]a and bilateral frontal hygroma [Figure 1]b. Two days later, brain MRI confirmed the SAH [Figure 1]c and the mild bilateral frontal hygroma [Figure 1]d and revealed diffuse pachymeningeal enhancement (DPE) [Figure 1]d. Cerebral angiography demonstrated normal cortical venous network and the absence of vascular malformations. The postcontrast whole spinal MRI showed no CSF leak sites and no seeding which ruled out meningeal carcinomatosis. Thoracic CT scan showed no lung cancer relapse. A diagnosis of SIH was made, according to The International Classification of Headache Disorders 3rd ed.ition (ICHD-3) criteria, and we treated the patient with conservative measures: Trendelenburg bed rest for three weeks, overhydration, and analgesic therapy, with progressive disappearance of his headache. One month later, brain CT scan showed cortical SAH disappearance. After four months, brain magnetic resonance imaging (MRI) [Figure 1]e revealed decreased DPE and frontal hygroma [Figure 1]f. The patient was asymptomatic at a 12-month follow-up and no recurrence of the tumor was found.
|Figure 1: At the beginning, brain CT scan in Emergency Room showed left frontal convexity SAH (a, arrowhead) and mild bilateral frontal hygroma (b, arrows). After 2 days, FLAIR MRI confirmed SAH (c, arrowhead); T1 postcontrast MRI showed the mild bilateral frontal hygroma and diffuse pachymeningeal enhancement (DPE) (d, arrowheads). Four months later, FLAIR MRI showed cortical SAH disappearance (e) and T1 MRI revealed decreased DPE and frontal hygroma (f)|
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A diagnosis of meningeal carcinomatosis was considered unlikely. In the latter condition, survival is very poor: worst in cases deriving from solid tumors (median 2.3 months) compared with 4.7 months for hematopoietic tumors patients. In about a quarter of cases, there is also an increase in intracranial pressure with the need to place a ventriculoperitoneal shunt. MRI plays an important role in the diagnosis of meningeal metastases, particularly in patients with solid tumors, probably because this kind of metastases has the propensity to form a nodular structure. In our patient, brain MRI showed symmetric and linear DPE, typical and specific sign of SIH, which is different from meningeal carcinomatosis conversely characterized by focal and/or nodular pachymeningeal and leptomeningeal enhancement, the latter absent in SIH. Other distinct abnormalities on brain MRI in SIH are as follows: subdural fluid collections (hygromas or hematomas), brain sagging, enlargement of pituitary, engorged cerebral venous sinuses, and decrease in size of the ventricles. Nontraumatic convexity SAH is uncommon and may be also associated with isolated cortical veins thrombosis from intracranial hypotension. Considering that a cortical venous thrombosis is missing in our case, as pathogenesis of the convexity SAH, it is possible to hypothesize that the prolonged and extreme venous dilatation caused by SIH might have caused a wall vessel tearing with consequent cortical SAH. The most typical symptom of SIH is orthostatic headache (in the upright position), relieved in recumbency.
In conclusion, SIH being a rare pathology still little known is often underestimated. Therefore, pachymeningeal thickening and enhancement of SIH, especially in patients with cancer, may be confused with a carcinomatous pachymeningitis with the risk of performing unnecessary, and sometimes invasive, examinations (such as lumbar puncture for neoplastic cells detection in CSF), and cycles of chemotherapy. Therefore, it is necessary to know the clinical and radiological features of SIH to make a correct differential diagnosis between SIH pachymeningitis and pachymeningeal carcinomatosis. According to our knowledge, this case is the first described of SIH in a patient with lung cancer.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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Clarke JL, Perez HR, Jacks LM, Panageas KS, DeAngelis LM. Leptomeningeal metastases in the MRI era. Neurology 2015;74:1449-54.
Ferrante E, Citterio A, Valvassori L, Arpino I, Tiraboschi P. A case of convexity subarachnoid haemorrhage from intracranial hypotension treated with epidural blood patch. Neurol Sci 2012;33:715-6.