Acta Neurol Belg DOI 10.1007/s13760-015-0448-6
NEURO-IMAGES
Bilateral middle cranial fossa arachnoidal cysts with temporal lobe agenesis associated with astrocytoma: fortuitous finding Boris Radic´ • Vladimir Miletic´ • Ratimir Petrovic´ Petra Radic´
•
Received: 8 February 2015 / Accepted: 17 February 2015 Ó Belgian Neurological Society 2015
Previously healthy, 36-year-old professional soldier presented with left-sided partial motor epileptic seizure with secondary generalisation. He denied alcohol consumption, any drug abuse, head trauma, recent infections and headaches. Initial examination was unremarkable and routine laboratory investigations were within normal range. Brain magnetic resonance imaging (MRI) showed large bilateral middle cranial fossa arachnoid cysts with compressive effect on adjacent neural structures, associated with infiltrative process in medial part of the right frontal lobe that affects cerebral cortex, subcortical and deep white matter, and spreads over the anterior part of the corpus callosum to the left side (Fig. 1). In addition, brain single-photon emission computed tomography (SPECT) with Tc-99m
B. Radic´ (&) Department of Neurology, Outpatient Clinic, University Hospital Centre Zagreb, Kisˇpatic´eva 12, 10000 Zagreb, Croatia e-mail: [email protected] V. Miletic´ Department of Neurology, Movement Disorders Centre, University Hospital Centre Zagreb, Kisˇpatic´eva 12, 10000 Zagreb, Croatia R. Petrovic´ Department of Nuclear Medicine and Radiation Protection, University Hospital Zagreb, Kisˇpatic´eva 12, 10000 Zagreb, Croatia P. Radic´ School of Medicine, University of Zagreb, Zagreb, Croatia
ECD was performed and showed diffuse areas of hypoperfusion in both frontal lobes together with the absence of radionuclide accumulation in both temporal lobes (Fig. 2). After stereotactic biopsy and pathohistological confirmation of anaplastic astrocytoma (WHO grade III), chemotherapy with lomustine (CCNU) and vincristine was started, followed by radiotherapy. In 1964, Robinson described ten cases with unilateral temporal lobe defects and coined the term ‘temporal lobe agenesis syndrome’ [1]. Bilateral temporal lobe agenesis associated with middle cranial fossa arachnoid cysts, on the other hand, is exceedingly a rare condition and only few cases have been reported so far [2, 3]. Aetiology of this syndrome is still a controversial subject. Original theory proposes that middle cranial fossa cysts represent a passive collection of cerebrospinal fluid related to temporal lobe agenesis [1], while second theory considers temporal lobe defects as a consequence of compression of arachnoid cysts as primary developmental malformation of the arachnoid membrane [4]. Most cases of middle cranial fossa cysts are asymptomatic. Some patients present with nonspecific symptoms and signs, including signs of elevated intracranial pressure, seizures, calvarial bulging, and focal neurological signs, while vision and hearing abnormalities, and behavioural changes are less common. In a recent retrospective study that included 48,417 patients who underwent brain MRI, middle cranial fossa arachnoid cysts were found in 237 patients (0.005 %); yet, cysts were considered to be symptomatic in only five patients [5]. In conclusion, although being impressive radiological finding, bilateral temporal lobe agenesis did not seem to have an impact on our patient’s physical and intellectual development and would probably remain unrevealed if the symptoms of associated malignancy did not occur.
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Acta Neurol Belg Fig. 1 a Sagittal MRI section, T1-weighted sequence showing bilateral middle cranial fossa arachnoidal cysts. b Transversal MRI section, T2-weighted sequence showing bilateral middle cranial fossa arachnoidal cysts and infiltrative process in the right frontal lobe spreading over the anterior part of the corpus callosum to the left side
Fig. 2 Single-photon emission computed tomography (SPECT) with Tc-99m ECD showing diffuse areas of hypoperfusion in both frontal lobes together with absence of radionuclide accumulation in both temporal lobes suggesting temporal lobe agenesis
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Acta Neurol Belg Conflict of interest of interest.
The authors declare that they have no conflict
Informed consent Written informed consent was obtained from the patient for publication of this case report and any accompanying electronic supplementary material.
References
2. Long C, Lehr S, Huk W (1981) A case of bilateral temporal lobe agenesis. J Neurol Neurosurg Psychiatr 44:626–630 3. Oztekin NS, Saygi SS, Oztekin F, Selekler K (1991) Bilateral middle cranial fossa arachnoid cysts. A case report. J Neurosurg Sci 35:153–156 4. Robinson RG (1971) Congenital cyst of the brain: arachnoid malformation. Prog Neurol Surg 4:133–174 5. Al-Holou WN, Terman S, Kilburg C, Garton HJ, Muraszko KM, Maher CO (2013) Prevalence and natural history of arachnoid cysts in adults. J Neurosurg 118:222–231
1. Robinson RG (1964) The temporal lobe agenesis syndrome. Brain 88:87–106
123
NEURO-IMAGES
Bilateral middle cranial fossa arachnoidal cysts with temporal lobe agenesis associated with astrocytoma: fortuitous finding Boris Radic´ • Vladimir Miletic´ • Ratimir Petrovic´ Petra Radic´
•
Received: 8 February 2015 / Accepted: 17 February 2015 Ó Belgian Neurological Society 2015
Previously healthy, 36-year-old professional soldier presented with left-sided partial motor epileptic seizure with secondary generalisation. He denied alcohol consumption, any drug abuse, head trauma, recent infections and headaches. Initial examination was unremarkable and routine laboratory investigations were within normal range. Brain magnetic resonance imaging (MRI) showed large bilateral middle cranial fossa arachnoid cysts with compressive effect on adjacent neural structures, associated with infiltrative process in medial part of the right frontal lobe that affects cerebral cortex, subcortical and deep white matter, and spreads over the anterior part of the corpus callosum to the left side (Fig. 1). In addition, brain single-photon emission computed tomography (SPECT) with Tc-99m
B. Radic´ (&) Department of Neurology, Outpatient Clinic, University Hospital Centre Zagreb, Kisˇpatic´eva 12, 10000 Zagreb, Croatia e-mail: [email protected] V. Miletic´ Department of Neurology, Movement Disorders Centre, University Hospital Centre Zagreb, Kisˇpatic´eva 12, 10000 Zagreb, Croatia R. Petrovic´ Department of Nuclear Medicine and Radiation Protection, University Hospital Zagreb, Kisˇpatic´eva 12, 10000 Zagreb, Croatia P. Radic´ School of Medicine, University of Zagreb, Zagreb, Croatia
ECD was performed and showed diffuse areas of hypoperfusion in both frontal lobes together with the absence of radionuclide accumulation in both temporal lobes (Fig. 2). After stereotactic biopsy and pathohistological confirmation of anaplastic astrocytoma (WHO grade III), chemotherapy with lomustine (CCNU) and vincristine was started, followed by radiotherapy. In 1964, Robinson described ten cases with unilateral temporal lobe defects and coined the term ‘temporal lobe agenesis syndrome’ [1]. Bilateral temporal lobe agenesis associated with middle cranial fossa arachnoid cysts, on the other hand, is exceedingly a rare condition and only few cases have been reported so far [2, 3]. Aetiology of this syndrome is still a controversial subject. Original theory proposes that middle cranial fossa cysts represent a passive collection of cerebrospinal fluid related to temporal lobe agenesis [1], while second theory considers temporal lobe defects as a consequence of compression of arachnoid cysts as primary developmental malformation of the arachnoid membrane [4]. Most cases of middle cranial fossa cysts are asymptomatic. Some patients present with nonspecific symptoms and signs, including signs of elevated intracranial pressure, seizures, calvarial bulging, and focal neurological signs, while vision and hearing abnormalities, and behavioural changes are less common. In a recent retrospective study that included 48,417 patients who underwent brain MRI, middle cranial fossa arachnoid cysts were found in 237 patients (0.005 %); yet, cysts were considered to be symptomatic in only five patients [5]. In conclusion, although being impressive radiological finding, bilateral temporal lobe agenesis did not seem to have an impact on our patient’s physical and intellectual development and would probably remain unrevealed if the symptoms of associated malignancy did not occur.
123
Acta Neurol Belg Fig. 1 a Sagittal MRI section, T1-weighted sequence showing bilateral middle cranial fossa arachnoidal cysts. b Transversal MRI section, T2-weighted sequence showing bilateral middle cranial fossa arachnoidal cysts and infiltrative process in the right frontal lobe spreading over the anterior part of the corpus callosum to the left side
Fig. 2 Single-photon emission computed tomography (SPECT) with Tc-99m ECD showing diffuse areas of hypoperfusion in both frontal lobes together with absence of radionuclide accumulation in both temporal lobes suggesting temporal lobe agenesis
123
Acta Neurol Belg Conflict of interest of interest.
The authors declare that they have no conflict
Informed consent Written informed consent was obtained from the patient for publication of this case report and any accompanying electronic supplementary material.
References
2. Long C, Lehr S, Huk W (1981) A case of bilateral temporal lobe agenesis. J Neurol Neurosurg Psychiatr 44:626–630 3. Oztekin NS, Saygi SS, Oztekin F, Selekler K (1991) Bilateral middle cranial fossa arachnoid cysts. A case report. J Neurosurg Sci 35:153–156 4. Robinson RG (1971) Congenital cyst of the brain: arachnoid malformation. Prog Neurol Surg 4:133–174 5. Al-Holou WN, Terman S, Kilburg C, Garton HJ, Muraszko KM, Maher CO (2013) Prevalence and natural history of arachnoid cysts in adults. J Neurosurg 118:222–231
1. Robinson RG (1964) The temporal lobe agenesis syndrome. Brain 88:87–106
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