Chronic lymphocytic infiltration with pontine perivascular ...
Pathological crying as a manifestation of spontaneous haemorrhage in a pontine cavernous haemangioma
2
Pathological crying as a manifestation of spontaneous haemorrhage in a pontine cavernous haemangioma Shradha Maheshwari a , Antonio Figueiredo a , Atul Goel a,b, * a Department of Neurosurgery, Lilavati Hospital and Research Centre, Bandra West, Mumbai, India b Department of Neurosurgery, Seth G. S. Medical College and King Edward Memorial Hospital, Acharya Donde Marg, Parel, Mumbai 400012, India article info Article history: Received 7 July 2009 Accepted 13 September 2009 Keywords: Brainstem cavernoma Haemorrhage Pathological crying abstract A 53-year-old man presented with a sudden onset severe headache associated with ataxia. His family observed labile emotions and frequent episodes of crying. Investigations revealed a pontine cavernoma with evidence of bleeding. The lesion was treated conservatively. Over approximately 6 months, the patient’s ataxia improved and he recovered from the crying episodes. To our knowledge, pathological cry- ing has not been previously associated with brainstem cavernoma. Ó 2009 Elsevier Ltd. All rights reserved. 1. Introduction Pathological laughter has been more often reported than path- ological crying with brainstem lesions. 1–5 However, patients who have had pathological laughter and crying occurring in unison and in isolation have both been reported and a causal relationship between the two has been established. We report a patient with a spontaneous haemorrhage in a pontine cavernous haemangioma who presented with pathological crying. 2. Case report A 53-year-old normotensive man presented with a sudden on- set occipital headache and ataxia. In addition, his relatives noted labile emotions including inappropriate, uncontrollable periods of crying, with each episode lasting 10–20 s. The episodes, which occurred in the absence of a stimulus, sometimes occurred more than 30 times per day. The patient appeared aware of his abnor- mal emotion and his consciousness was not impaired during these episodes. His psychiatric history and conventional neuro- psychological assessment were unremarkable. Examination did not reveal any emotional or intellectual disturbance. Neurological examination showed right-side finger–nose ataxia and mild dysdiadochokinesia. Romberg’s test was positive. There were no eye signs, long tract signs or cranial nerve deficits. Investigations revealed characteristic features of bleeding within a relatively large pontine cavernoma (Figs. 1, 2). No surgery was advised at this time. The patient’s headaches and ataxia rapidly improved. The crying episodes also progressively and sequentially became fewer and resolved after 6 months. A repeat MRI after 6 months showed regression in the size of lesion with resorption of the clot (Fig. 3). 3. Discussion Laughing and crying are termed pathological when the behav- iour is inappropriate or exaggerated, and occurs without an appar- ent motivating stimulus or in response to stimuli that would not normally produce this emotional reaction. The absence of co-exist- ing emotional changes always signifies a pathological substrate, either diffuse or focal, in the brain. 6 Pathological laughter is consid- erably more common than pathological crying. It appears that the two responses represent discrete symptoms and the site of the causative lesion is also probably different. However, there have been reports of patients in whom both crying and laughing occurred. 7 Normal crying is a co-ordinated motor function involving facial and respiratory muscles, is triggered by specific stimuli, and accompanied by mood depression. Paroxysmal attacks of crying that are uncontrollable, involuntary, occur in the absence of con- gruent mood changes, and are precipitated by minor affective or neutral events are termed pathological. Pathological crying has been observed in patients with lesions involving diverse areas of the brain, including the cortex, diencephalon and brainstem; how- ever in most, lesions involving systems affecting motor function (pyramidal or extrapyramidal fibres) and interruption at the brain- stem has been observed. 6,8 The differential diagnosis of pathologi- cal crying in patients with brainstem and cerebellar lesions is mood disorder, such as crying related to depression. The differen- tiation between the two conditions may not always be easy. 9 Assessment scales have been developed specifically for character- izing pathological crying and pathological laughter, although these were not necessary for our patient. 10,11 The precise mechanism and neuroanatomy of pathological crying remains uncertain. Parvizi et al. explored and discussed the pathophysiology of pathological crying and postulated that the lesion occurs in the cerebro-ponto-cerebellar pathways. 12 They postulated a disruption of the pathway from higher associ- ation areas to the cerebellum, resulting in the cerebellum failing to co-ordinate and adjust the crying response to the appropriate cognitive and situational context. The haemorrhage from the cavernoma probably disrupted the cerebro-pontine-cerebellar pathway. Our patient had cerebellar symptoms; therefore, the le- sion probably involved the cerebellar peduncle. Some researchers have speculated that such lesions probably occur above the level of the facial nuclei and respiratory centre. The prevalence of these conditions is thought to be about 5–10% among patients with various conditions such as traumatic brain injury, 13 multiple sclerosis, 3–5,7 amyotrophic lateral sclerosis, Parkinson’s disease, subcortical and brainstem infarction, 1,2,14 and tumours in and around the brainstem. 15,16 Our literature search did not reveal any patients in whom pathological crying was associated with cavernoma of the brainstem. * Corresponding author. Tel.: +91 22 24129884; fax: +91 22 24143435. E-mail address: [email protected] (A. Goel). 662 Case Reports / Journal of Clinical Neuroscience 17 662–663
-
Upload
shradha-maheshwari -
Category
Documents
-
view
212 -
download
0
Transcript of Pathological crying as a manifestation of spontaneous haemorrhage in a pontine cavernous haemangioma
662 Case Reports / Journal of Clinical Neuroscience 17 662–663
Pathological crying as a manifestation of spontaneous haemorrhagein a pontine cavernous haemangioma
Shradha Maheshwari a, Antonio Figueiredo a, Atul Goel a,b,*
a Department of Neurosurgery, Lilavati Hospital and Research Centre, Bandra West, Mumbai, Indiab Department of Neurosurgery, Seth G. S. Medical College and King Edward Memorial Hospital, Acharya Donde Marg, Parel, Mumbai 400012, India
a r t i c l e i n f o
Article history:Received 7 July 2009Accepted 13 September 2009
Keywords:Brainstem cavernomaHaemorrhagePathological crying
a b s t r a c t
A 53-year-old man presented with a sudden onset severe headache associated with ataxia. His familyobserved labile emotions and frequent episodes of crying. Investigations revealed a pontine cavernomawith evidence of bleeding. The lesion was treated conservatively. Over approximately 6 months, thepatient’s ataxia improved and he recovered from the crying episodes. To our knowledge, pathological cry-ing has not been previously associated with brainstem cavernoma.
� 2009 Elsevier Ltd. All rights reserved.
1. Introduction
Pathological laughter has been more often reported than path-ological crying with brainstem lesions.1–5 However, patients whohave had pathological laughter and crying occurring in unisonand in isolation have both been reported and a causal relationshipbetween the two has been established. We report a patient with aspontaneous haemorrhage in a pontine cavernous haemangiomawho presented with pathological crying.
2. Case report
A 53-year-old normotensive man presented with a sudden on-set occipital headache and ataxia. In addition, his relatives notedlabile emotions including inappropriate, uncontrollable periods ofcrying, with each episode lasting 10–20 s. The episodes, whichoccurred in the absence of a stimulus, sometimes occurred morethan 30 times per day. The patient appeared aware of his abnor-mal emotion and his consciousness was not impaired duringthese episodes. His psychiatric history and conventional neuro-psychological assessment were unremarkable. Examination didnot reveal any emotional or intellectual disturbance. Neurologicalexamination showed right-side finger–nose ataxia and milddysdiadochokinesia. Romberg’s test was positive. There were noeye signs, long tract signs or cranial nerve deficits. Investigationsrevealed characteristic features of bleeding within a relativelylarge pontine cavernoma (Figs. 1, 2). No surgery was advised atthis time. The patient’s headaches and ataxia rapidly improved.The crying episodes also progressively and sequentially becamefewer and resolved after 6 months. A repeat MRI after 6 monthsshowed regression in the size of lesion with resorption of the clot(Fig. 3).
3. Discussion
Laughing and crying are termed pathological when the behav-iour is inappropriate or exaggerated, and occurs without an appar-ent motivating stimulus or in response to stimuli that would notnormally produce this emotional reaction. The absence of co-exist-ing emotional changes always signifies a pathological substrate,
* Corresponding author. Tel.: +91 22 24129884; fax: +91 22 24143435.E-mail address: [email protected] (A. Goel).
either diffuse or focal, in the brain.6 Pathological laughter is consid-erably more common than pathological crying. It appears that thetwo responses represent discrete symptoms and the site of thecausative lesion is also probably different. However, there havebeen reports of patients in whom both crying and laughingoccurred.7
Normal crying is a co-ordinated motor function involving facialand respiratory muscles, is triggered by specific stimuli, andaccompanied by mood depression. Paroxysmal attacks of cryingthat are uncontrollable, involuntary, occur in the absence of con-gruent mood changes, and are precipitated by minor affective orneutral events are termed pathological. Pathological crying hasbeen observed in patients with lesions involving diverse areas ofthe brain, including the cortex, diencephalon and brainstem; how-ever in most, lesions involving systems affecting motor function(pyramidal or extrapyramidal fibres) and interruption at the brain-stem has been observed.6,8 The differential diagnosis of pathologi-cal crying in patients with brainstem and cerebellar lesions ismood disorder, such as crying related to depression. The differen-tiation between the two conditions may not always be easy.9
Assessment scales have been developed specifically for character-izing pathological crying and pathological laughter, although thesewere not necessary for our patient.10,11
The precise mechanism and neuroanatomy of pathologicalcrying remains uncertain. Parvizi et al. explored and discussedthe pathophysiology of pathological crying and postulated thatthe lesion occurs in the cerebro-ponto-cerebellar pathways.12
They postulated a disruption of the pathway from higher associ-ation areas to the cerebellum, resulting in the cerebellum failingto co-ordinate and adjust the crying response to the appropriatecognitive and situational context. The haemorrhage from thecavernoma probably disrupted the cerebro-pontine-cerebellarpathway. Our patient had cerebellar symptoms; therefore, the le-sion probably involved the cerebellar peduncle. Some researchershave speculated that such lesions probably occur above the levelof the facial nuclei and respiratory centre. The prevalence of theseconditions is thought to be about 5–10% among patients withvarious conditions such as traumatic brain injury,13 multiplesclerosis,3–5,7 amyotrophic lateral sclerosis, Parkinson’s disease,subcortical and brainstem infarction,1,2,14 and tumours in andaround the brainstem.15,16 Our literature search did not revealany patients in whom pathological crying was associated withcavernoma of the brainstem.
Fig. 3. Sagittal T1-weighted MRI, 6 months after ictus, showing partial resorption ofthe haematoma within the cavernoma.
Fig. 1. Axial CT scan showing the pontine cavernoma.
Fig. 2. Sagittal T1-weighted MRI showing a relatively large pontine lesion withsurrounding haemorrhage.
Case Reports / Journal of Clinical Neuroscience 17 662–663 663
4. Conclusion
Pontine lesions may be suspected in patients for whom the pre-senting symptom is pathological crying.
doi:10.1016/j.jocn.2009.09.009
References
1. Anderson G, Ingeman-Neilsen M, Vestergaard K, et al. Pathoanatomiccorrelation between post stroke pathological crying and damage tobrain areas involved in serotonergic neurotransmission. Stroke 1994;25:1050–2.
2. Garg RK, Misra S, Verma R. Pathological laughter as heraldingmanifestation of left middle cerebral artery territory infarct. NeurolIndia 2000;48:388–90.
3. Matsuoka S, Vakota A, Yasukouchi H, et al. Clival chordoma associated withpathological laughter: case report. J Neurosurg 1993;79:428–33.
4. Muzumdar D, Agrahar P, Desai K, et al. Pathological laughter as a presentingsymptom of petroclival meningioma: case report. Neurol Med-Chir (Tokyo)2001;41:505–7.
5. Tei H, Sakamoto Y. Pontine infarction due to basilar artery infarction presentingas pathological laughter. Neuroradiology 1997;39:190–1.
6. Arlazaroff A, Mester R, Spirak B, et al. Pathological laughter: common vsunusual aetiology and presentation. J Psychiatry Relat Sci 1998;35:184–9.
7. Virani MJ, Jain S. Trigeminal schwanoma associated with pathological cryingand laughter. Neurol India 2001;49:162–5.
8. Parvizi J, Coburn KL, Shillcutt SD, et al. Neuroanatomy of pathological laughingand crying: a report of the American Neuropsychiatric Association Committeeon Research. J Neuropsychiatry Clin Neurosci 2009;21:75–87.
9. Parvizi J, Arciniegas DB, Bernardini GL, et al. Diagnosis and management ofpathological laughter and crying. Mayo Clin Proc 2006;81:1482–6.
10. Smith RA, Berg JE, Pope LE, et al. Validation of the CNS emotional lability scalefor pseudobulbar affect (pathological laughter and crying) in multiple sclerosispatients. Mult Scler 2004;10:679–85.
11. Robinson RG, Parikh RM, Lipsey JR, et al. Pathological laughter and cryingfollowing stroke: validation of measurement scale and double blind treatmentstudy. Am J Psychiatry 1993;150:286–93.
12. Parvizi J, Anderson SW, Martin CO, et al. Pathological laughter and crying. A linkto the cerebellum. Brain 2001;124:1708–19.
13. Zeilig G, Drubach DA, Katz-Zeilig M, et al. Pathological laughter andcrying in patients with closed traumatic brain injury. Brain Inj 1996;10:591–7.
14. Kosaka H, Omata M, Omori M, et al. Abnormal pontine activation inpathological laughing as shown by functional magnetic resonance imaging.J Neurol Neurosurg Psychiatry 2006;77:1376–80.
15. Bhaskar IP, Chacko AG, Daniel RT. Brainstem compression from a trigeminalschwannoma presenting with a pathological crying. J Clin Neurosci2008;15:322–4.
16. Parvizi J, Schiffer R. Exaggerated crying and tremor with a cerebellar cyst.J Neuropsychiatry Clin Neurosci 2007;19:187–90.
