A young patient who presented with headache followed by positive and negative symptoms
of psychosis and mutism was sent for the MRI of brain. MRI revealed a lipoma in the
quardrigeminal area. We hypothesized that the neuro-vascular encasement of structures
located at the upper dorsal midbrain by the lipoma caused the symptoms. A review of
the current literature of quadrigeminal lipoma cases with presenting symptoms is provided.
Lipoma in quardrigeminal area could give rise to symptoms of psychosis.
Intracranial lipomas are extremely rare benign tumors, accounting for <0.1 % of all
primary brain neoplasms (1). Most commonly, they occur in the trigonal choroid plexus
on cerebral convexities in the pericallosal area, suprasellar/interpeduncular cistern,
cerebellopontine angle cistern, sylvian cistern and in quadrigeminal cistern regions
(2, 3). Most of them are asymptomatic, usually discovered as incidental findings during
autopsy or brain imaging (4). Rarely, they may be symptomatic and found to be associated
with seizures or headache (5). Here, we present a case of a teenage female presenting
with symptoms of psychosis whose MRI scanning showed a quadrigeminal cistern lipoma.
We believe that this is the first reported case ever of such a lesion presenting with
psychosis.
Case report
The case was a 17- year- old unmarried female patient studying in 9th standard from
a low socio-economic background without any significant past or family history presented
with symptoms of irrelevant talks, muttering, smiling to self without any apparent
reason, disorganised behaviour and poor self-care for the last one month.
Her symptoms started gradually three months ago when she complained of persistent,
constricting type of diffuse headache of mild to moderate severity, which increased
when she was reading. Two weeks later, she woke up one night with a sudden cry and
shouting, expressing that she felt pain all over her body as if something was pressing
on her. She became restless, fearful and wandered aimlessly in the house for hours.
However, her consciousness was never altered and she never became unresponsive even
for a brief period of time.
Over the next few days, her restlessness continued to increase, and she became impulsive
and touched and pulled others coming near her without any apparent reason. Her sleep
was decreased and she showed odd behaviours like nodding her head, shaking the hands
and head as well as making odd gestures. Smiling to self and drooling of saliva were
also noticed, and her self-care decreased. She also micturated and defecated in her
clothes and did not seem disturbed about it. There was a general loss of shamefulness
and on multiple occasions she took out her clothes in public or wandered around inadequately
dressed. Gradually, her talking decreased to the point of being silent.
There was some improvement in her symptoms over the next two- three weeks spontaneously
without any treatment. She started talking, although mostly irrelevantly, and was
not found wandering with inadequate clothes though her clothing remained mostly dirty
and dishevelled. However, her self-care continued to remain poor, she smiled to herself
and muttered on occasions. She also micturated or defecated outside her house though
ablution was limited and unsatisfactory. She was also noted to be sitting or standing
in the same position for long durations and did not respond adequately when enquired
about the same.
There was no history to suggest seizures, delirium, drug intoxication, delusions,
bipolar disorder, dissociative disorders, sleep disorders and any medical or surgical
disorders.
Mental status examination revealed clear consciousness. Her attention could be aroused
easily but it was ill-sustained, and there was mild impairment in orientation. The
patient was comprehending communication, as was evident by her following instructions
like looking at her mother, picking up the pen etc. However, her judgement was impaired.
Table1
Clinical presentation of quadrigeminal cistern lipomas
Year
Author(s)
n
Age (in Years) /Sex
Country
Symptoms
2013
Jha et al (12)
1
3
India
single episode of generalized tonic-clonic seizures
2013
Majumdar et al (13)
1
10M
India
headache since 2 years of age along with recurrent vomiting and drooping of left eyelid
during the attack
2012
Khoshnevisan et al (14)
1
20M
Iran
Headache
2012
Panil Kumar et al (15)
1
32M
India
Headache and Seizures
2009
Ogbole et al (1)
1
70F
Nigeria
Headache
2008
Senoglu & Altun (7)
1
37F
Turkey
Headache
2005
Yilmazlar et al (11)
1
37F
Turkey
raised intracranial pressure
2005
Fandiño et al (4)
1
47
Spain
headache dizziness and quadrantanopsia
2002
Kiymaz & Cirak (16)
1
2F
Turkey
encephalocraniocutaneous lipomatosis
1998
Ono et al (3)
1
7M
Japan
complex partial seizures
1998
Sala et al (17)
1
4M
Italy
Epilepsy and behavioural change
1995
Nikaido et al (8)
1
65M
Japan
left abducens nerve paresis
1993
Uchino et al (18)
6
Japan
mildly dilated ventricular system in one, rest asymptomatic
1991
Howng & Chang (19)
1
China
headache; especially over the occipital area; and, blurring of vision
1989
Uchino et al (20)
1
Japan
Asymptomatic
1987
Summers et al (21)
1
10F
USA(Minneapolis)
Congenital ocular motor apraxia
1987
Maiuri et al (22)
1
62M
Italy
Intracranial hypertension
1986
Friedman et al (23)
1
63M
USA(Maryland)
Headache, blurred vision, behavioural change
1985
Ambrosetto et al (24)
2
Italy
impairment of vertical gaze in one
1983
Hayashi et al (25)
2
infants
Japan
obstructive hydrocephalus in one, agenesis of corpus callosum in other
Fig 1
MRI Brain showing lipoma in the quardrigeminal area
1A. T1 image showing normal sized lateral ventricles
1B. T1 image showing lipoma in quardrigeminal area
1C. T2 image showing lipoma in quardrigeminal area
Her hygiene was poor with dishevelled clothes, unkempt hair, restlessness, distractibility
and poor maintenance of eye contact. Her speech was hesitant and mostly irrelevant
with loosening of associations, occasionally incoherent and was barely audible. Her
affect was inappropriate and labile. Physical examination revealed mildly brisk deep
tendon reflexes (bilateral biceps, supinator, knee and ankle jerks were Grade 3+)
and equivocal bilateral planter response. Further examination was not possible.
The patient was seen by the consultant psychiatrist of the day and was diagnosed with
acute and transient psychotic disorder as per International Statistical Classification
of Diseases and Related Health Problems (ICD)- 10th Revision criteria (6). Her routine
blood investigations and brain MRI (without contrast) were ordered. All the routine
blood investigations were within the normal limits. MRI scan of the brain, however,
showed a lesion (0.5cm x 0.42cm x 0.45cm) in the quadrigeminal cistern area in the
posterior aspect of the right inferior colliculus of the midbrain and right superior
medullary vellum and anterior to the lingula of the right cerebellar hemisphere. The
lesion was hyperintense in both T1 and T2 sequences which was diagnosed as lipoma
since these signal intensities were consistent with fat (7). However, fat suppression
sequence in MRI could not be performed as it is not done routinely for all cases in
our centre due to the very high workload. There was no evidence of any mass effect
or obstructive changes in the brain parenchyma. Neurosurgical referral was sought,
where conservative management was recommended. The patient was prescribed Tab. Olanzapine
5mg per day (increased to 10 mg per day after 5 days) and 1 mg of tab. Lorazepam,
to which she responded within one month, and is currently on the same medication.
Discussion
The patient was diagnosed as a case of psychosis due to the presence of disorganized
behaviour, formal thought disorder, persecutory ideas/delusions and negative symptoms
in the form of abulia and poverty of speech (6). Moreover, keeping with the nature
of acute and transient psychosis, it also presented with a polymorphic course. Delirium
and focal seizure were ruled out by absence of alteration in consciousness, presence
of adequate responsiveness and prolonged symptoms. However, some notable findings
of the case were headache at the onset, slowly developing and variable mutism, a sensation
described by the patient as “as if something is pressing” and soiling of clothes with
urine and stool.
Headache preceding the onset of psychosis along with soiling of clothes necessitated
the brain imaging in this case. The incontinence could be the urge or overflow incontinence,
or it could be a part of disorganized behavior of psychosis. The “as if something
is pressing” sensation in the absence of any stimulus is likely to be tactile hallucination.
Explanation for both of these symptoms could not be clarified beyond doubt due to
the difficulty in communicating with the patient.
The imaging findings confirmed lipoma of the quadrigeminal plate. Earlier studies
have reported that further histo-pathological confirmation is not necessary to diagnose
lipoma; hence, they were not sought (7). There are other differential diagnosis for
lipoma in this region which include arachnoid and tectal plate cyst, tectal masses,
supracerebellar abscess, dermoid and epidermoid cysts, ruptured P4 segment aneurysm
of the posterior cerebral artery and also pineal region mass (8,1).
Intracranial lipomas are extremely rare developmental tumors arising from abnormal
persistence and development of primitive meninges (9). Lipoma in the quadrigeminal
region includes that in the quadrigeminal cistern, the quadrigeminal plate, the ambient
cistern, the superior vermis, or the superior medullary velum (1, 10). In about one
fifth of the cases, these lipomas can cause significant mass effects (3), with neurologic
deficits, obstructive hydrocephalus, or raised intracranial pressure (11). Usually
patients present with headache, dizziness, psychomotor retardation, generalized or
complex partial seizures, visual disturbances or may be asymptomatic (8). A review
of literature of reported cases of quadrigeminal lipoma with presenting symptoms is
given in Table 1. As of yet, there are no such case reports of subjects presenting
with psychosis in association with such a lipoma.
Lipomas rarely compress or displace the adjacent neural tissue, and they have been
hypothesized to encase the nerves and vessels involving the surrounding structures
to give rise to a variety of symptoms (17). Symptoms like headache, seizures, loss
of consciousness, cranial nerve palsy, behavioral abnormality including aggressive
behavior have been attributed to similar quadrigeminal lipomas without any pressure
or mass effect in previous case reports (1, 7, 8, 12 and 17). Behavioral changes including
aggressive behavior in quadrigeminal cistern lipoma have been hypothesized due to
involvement of midbrain- limbic system of Nauta (17, 26). It includes several structures
located at the upper dorsal midbrain including mesencephalic reticular system, the
periventricular grey matter, and the midbrain-limbic system (17, 27). Moreover, mutism
has also been reported in epidermoid cyst in the quadrigeminal cistern region in the
literature, suggesting that some anatomical substrate is present in this area that
can induce such symptoms (28). Visceral (Tactile) hallucinations have been reported
in a patient with tumor of thalamus (29), which is anatomically near the current site
of the lesion. In this case, we hypothesized that the appearance of psychotic symptoms
is due to similar entrapment of neural tissue by the quadrigeminal plate lipoma.
However, since surgical removal of the lipoma was not planned in this case, due to
the absence of any compressive effect and difficulty in operating such tumors due
to ensheathment of the surrounding tissues, the exact cause effect relationship of
the tumor with the symptoms could not be commented upon with certainty. The fact that
the symptoms responded to medical treatment does not prove the absence of any relation
between the lipoma and the symptoms, as earlier reports indicated psychotorpics are
effective in treating psychotic symptoms and catatonia with identifiable medical or
neurological illness (30). Previous reports of headache, loss of consciousness, seizures
attributed to similar lipomas have also responded well to medical management (1, 7,
12 and 17). On the contrary, previously reported oculo-motor apraxia and seizures
due to similar lipomas remained unchanged even after total or partial removal of the
tumor (21, 31). Therefore, the relationship between intracranial lipomas and its symptoms
may not be as linear as that of a mass effect relationship. The idea behind reporting
this case is to add to the body of the literature a very rare condition with an even
more atypical associated presentation.