Acta Neurol Belg DOI 10.1007/s13760-015-0470-8
NEURO-IMAGES
A frontal syndrome due to a brainstem lesion T. Decramer1 • Ph. Demaerel2 • R. Lemmens3,4,5
Received: 18 March 2015 / Accepted: 27 March 2015 Ó Belgian Neurological Society 2015
Keywords Frontal syndrome Brainstem Metastasis Lung cancer Neurobehavioral A 48-year-old man presented with progressive gait instability over a period of several weeks, during which he sustained a right forearm and right patellar fracture. Neurological examination revealed a vertical nystagmus in all gaze directions, severe slurring of speech, left sided hemiataxia and extensor plantar response. Computed tomography (CT) revealed a large inhomogeneous brainstem lesion with some spontaneous hyperdensity (Fig. 1a, axial CT without contrast). Magnetic resonance imaging (MRI) confirmed a large contrast-enhancing lesion in the lower part of the mesencephalon (Fig. 1b–d) and a second smaller lesion in the left cerebellar hemisphere. Combined CT chest/abdomen revealed a large primary lesion in the left lung and a left adrenal metastasis. Based on these findings, a tentative diagnosis of metastatic lung cancer & T. Decramer [email protected] Ph. Demaerel [email protected] R. Lemmens [email protected] 1
Department of Neurosurgery, University Hospitals Leuven, Herestraat 49, 3000 Louvain, Belgium
2
Department of Radiology, University Hospitals Leuven, Louvain, Belgium
3
Department of Neurosciences, Experimental Neurology, KU Leuven-University of Leuven, 3000 Louvain, Belgium
4
VIB-Vesalius Research Center, Louvain, Belgium
5
Department of Neurology, University Hospitals Leuven, 3000 Louvain, Belgium
was made which was further investigated by a percutaneous biopsy of the lung lesion confirming the presence of a large-cell adenocarcinoma. Lung cancer is the most common primary tumor (40–50 %) to metastasize to the brain, followed by breast (15–20 %), skin (5–10 %) and gastrointestinal (4–6 %) tumors [1]. Without treatment, the median survival of patients with brain metastasized lung cancer is 4–7 weeks [2]. The prognosis of this particular patient was extremely poor given the delicate brainstem localization. Several days after admission loss of decorum was noticed and episodes of aggression occurred which were difficult to control. Repeated imaging of the brain excluded hemorrhage or hydrocephalus as a potential cause for the clinical deterioration; the brainstem lesion had slightly grown. We hypothesized these psychiatric symptoms to arise as a consequence of the brainstem lesion. Two rare psychiatric conditions are known to be caused by brainstem lesions. One of them is peduncular hallucinosis (PH), a condition in which the patient suffers vivid and colorful visual hallucinations due to a, typically vascular, lesion in the mesencephalon or thalamus [3]. Although behavioral abnormalities can be seen in PH, the presence of hallucinations is a hallmark feature. A second condition is called pseudobulbar affect (PBA), a disinhibition syndrome in which the patient suffers from strong emotional lability with uncontrollable episodes of crying and/or laughing [4]. We believe our patient had a clinically distinct condition. Neurobehavioral effects of brainstem lesions have been reported, mimicking clinical features of frontal lobe pathology [5]. This is likely due to disruption of ascending neurotransmitter pathways projecting to the frontal and limbic cortex. The importance of ascending pathways such as the reticular activating system in modifying alertness is well described. The brainstem,
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Acta Neurol Belg
prior to admission, we believe a diagnosis of steroid induced psychosis is less likely. Following these behavioral changes his motor function rapidly decreased and there was no response to corticosteroids started on admission. Due to the severity of the condition and clinical deterioration, no further therapy was initiated after consulting the patient and his family; he died 2 weeks after admission. Autopsy was not performed. Conflict of interest
We report no conflicts of interest.
Ethical Standard We hereby declare that we acted in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki. Informed consent for publication was impossible to obtain from the patient because he deceased several weeks after admission. We didn’t include any personal details, so this case report is anonymous.
References Fig. 1 a Axial CT without contrast shows a hyperdense lesion in the lower part of the mesencephalon and pons, b axial T1 weighted MRI without contrast shows a hypointense lesion, c, d T1 weighted MRI with gadolinium shows a large contrast-enhancing lesion sagittal and axial respectively
however, is not conventionally considered to have a role in higher brain functions and behavior. Alternatively his behavioral changes could be caused by a syndrome called steroid psychosis, due to the high-dose corticosteroids started on admission. The presentation of this syndrome is variable and unpredictable. Steroid psychosis is actually a misnomer because it can also cause delirium, confusion, insomnia, emotional lability, depression and mania [6]. Loss of decorum, however, has not been reported in steroid psychosis. Since his medical history revealed subtle changes in behavior several weeks
123
1. Lassman AB, DeAngelis LM (2003) Brain metastases. Neurol Clin 21(1):1–23 (vii) 2. Chi A, Komaki R (2010) Treatment of brain metastasis from lung cancer. Cancers 2:2100–2137. doi:10.3390/cancers2042100 (published Online First: Epub Date) 3. Dogan VB, Dirican A, Koksal A (2013) A case of peduncular hallucinosis presenting as a primary psychiatric disorder. Ann Indian Acad Neurol 16:684–686. doi:10.4103/0972-2327.120469 (published Online First: Epub Date) 4. Miller A, Pratt H, Schiffer RB (2011) Pseudobulbar affect: the spectrum of clinical presentations, etiologies and treatments. Expert Rev Neurother 11:1077–1088 (published Online First: Epub Date) 5. Omar R, Warren JD, Ron MA (2007) The neuro-behavioural syndrome of brainstem disease. Neurocase 13:452–465. doi:10. 1080/13554790802001403 (published Online First: Epub Date) 6. Ross DA, Cetas JS (2012) Steroid psychosis: a review for neurosurgeons. J neuro Oncol 109:439–447. doi:10.1007/s11060012-0919-z (published Online First: Epub Date)
NEURO-IMAGES
A frontal syndrome due to a brainstem lesion T. Decramer1 • Ph. Demaerel2 • R. Lemmens3,4,5
Received: 18 March 2015 / Accepted: 27 March 2015 Ó Belgian Neurological Society 2015
Keywords Frontal syndrome Brainstem Metastasis Lung cancer Neurobehavioral A 48-year-old man presented with progressive gait instability over a period of several weeks, during which he sustained a right forearm and right patellar fracture. Neurological examination revealed a vertical nystagmus in all gaze directions, severe slurring of speech, left sided hemiataxia and extensor plantar response. Computed tomography (CT) revealed a large inhomogeneous brainstem lesion with some spontaneous hyperdensity (Fig. 1a, axial CT without contrast). Magnetic resonance imaging (MRI) confirmed a large contrast-enhancing lesion in the lower part of the mesencephalon (Fig. 1b–d) and a second smaller lesion in the left cerebellar hemisphere. Combined CT chest/abdomen revealed a large primary lesion in the left lung and a left adrenal metastasis. Based on these findings, a tentative diagnosis of metastatic lung cancer & T. Decramer [email protected] Ph. Demaerel [email protected] R. Lemmens [email protected] 1
Department of Neurosurgery, University Hospitals Leuven, Herestraat 49, 3000 Louvain, Belgium
2
Department of Radiology, University Hospitals Leuven, Louvain, Belgium
3
Department of Neurosciences, Experimental Neurology, KU Leuven-University of Leuven, 3000 Louvain, Belgium
4
VIB-Vesalius Research Center, Louvain, Belgium
5
Department of Neurology, University Hospitals Leuven, 3000 Louvain, Belgium
was made which was further investigated by a percutaneous biopsy of the lung lesion confirming the presence of a large-cell adenocarcinoma. Lung cancer is the most common primary tumor (40–50 %) to metastasize to the brain, followed by breast (15–20 %), skin (5–10 %) and gastrointestinal (4–6 %) tumors [1]. Without treatment, the median survival of patients with brain metastasized lung cancer is 4–7 weeks [2]. The prognosis of this particular patient was extremely poor given the delicate brainstem localization. Several days after admission loss of decorum was noticed and episodes of aggression occurred which were difficult to control. Repeated imaging of the brain excluded hemorrhage or hydrocephalus as a potential cause for the clinical deterioration; the brainstem lesion had slightly grown. We hypothesized these psychiatric symptoms to arise as a consequence of the brainstem lesion. Two rare psychiatric conditions are known to be caused by brainstem lesions. One of them is peduncular hallucinosis (PH), a condition in which the patient suffers vivid and colorful visual hallucinations due to a, typically vascular, lesion in the mesencephalon or thalamus [3]. Although behavioral abnormalities can be seen in PH, the presence of hallucinations is a hallmark feature. A second condition is called pseudobulbar affect (PBA), a disinhibition syndrome in which the patient suffers from strong emotional lability with uncontrollable episodes of crying and/or laughing [4]. We believe our patient had a clinically distinct condition. Neurobehavioral effects of brainstem lesions have been reported, mimicking clinical features of frontal lobe pathology [5]. This is likely due to disruption of ascending neurotransmitter pathways projecting to the frontal and limbic cortex. The importance of ascending pathways such as the reticular activating system in modifying alertness is well described. The brainstem,
123
Acta Neurol Belg
prior to admission, we believe a diagnosis of steroid induced psychosis is less likely. Following these behavioral changes his motor function rapidly decreased and there was no response to corticosteroids started on admission. Due to the severity of the condition and clinical deterioration, no further therapy was initiated after consulting the patient and his family; he died 2 weeks after admission. Autopsy was not performed. Conflict of interest
We report no conflicts of interest.
Ethical Standard We hereby declare that we acted in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki. Informed consent for publication was impossible to obtain from the patient because he deceased several weeks after admission. We didn’t include any personal details, so this case report is anonymous.
References Fig. 1 a Axial CT without contrast shows a hyperdense lesion in the lower part of the mesencephalon and pons, b axial T1 weighted MRI without contrast shows a hypointense lesion, c, d T1 weighted MRI with gadolinium shows a large contrast-enhancing lesion sagittal and axial respectively
however, is not conventionally considered to have a role in higher brain functions and behavior. Alternatively his behavioral changes could be caused by a syndrome called steroid psychosis, due to the high-dose corticosteroids started on admission. The presentation of this syndrome is variable and unpredictable. Steroid psychosis is actually a misnomer because it can also cause delirium, confusion, insomnia, emotional lability, depression and mania [6]. Loss of decorum, however, has not been reported in steroid psychosis. Since his medical history revealed subtle changes in behavior several weeks
123
1. Lassman AB, DeAngelis LM (2003) Brain metastases. Neurol Clin 21(1):1–23 (vii) 2. Chi A, Komaki R (2010) Treatment of brain metastasis from lung cancer. Cancers 2:2100–2137. doi:10.3390/cancers2042100 (published Online First: Epub Date) 3. Dogan VB, Dirican A, Koksal A (2013) A case of peduncular hallucinosis presenting as a primary psychiatric disorder. Ann Indian Acad Neurol 16:684–686. doi:10.4103/0972-2327.120469 (published Online First: Epub Date) 4. Miller A, Pratt H, Schiffer RB (2011) Pseudobulbar affect: the spectrum of clinical presentations, etiologies and treatments. Expert Rev Neurother 11:1077–1088 (published Online First: Epub Date) 5. Omar R, Warren JD, Ron MA (2007) The neuro-behavioural syndrome of brainstem disease. Neurocase 13:452–465. doi:10. 1080/13554790802001403 (published Online First: Epub Date) 6. Ross DA, Cetas JS (2012) Steroid psychosis: a review for neurosurgeons. J neuro Oncol 109:439–447. doi:10.1007/s11060012-0919-z (published Online First: Epub Date)
