'It's not just anger . It's Bipolar II'

We live in an era that now pathologizes all

types of human behaviours. For example,

what was once essentially the purview of

the criminal justice system has gradually

etched its way into mental health: anger

and violence.

The psychiatric literature is currently

abound with ’explanations’ such as neuro-

transmitter derangements, gene abnormal-

ities and now magnetic resonance imaging

scans to help themental health professional

understand and treat the disorder that

anger has become. Anger itself has now

assumed medical pseudonyms such as

mood swings, intermittent explosive disor-

der and rage attacks. Let us not forget the

much cherished bipolar disorder – which

has become the explanation for virtually

every act of domestic violence and public

brawling– inwhich thepublic finds itself the

central destructive actor. Anger has

increasingly become a no-fault condition,

whereby the notion of personal responsi-

bility is eschewed in favour of ’a chemical

imbalance’.

My own patients are uttering these

terms faster than I can pronounce my own

name. Yet, every time I explore what they

actually mean by ’mood swings’, invari-

ably I find that they are describing

irritability rather than lability. The ques-

tion I ask myself is: have clinicians

’forgotten’ to make this distinction? Does

it really matter? Call me old-fashioned but

is not that the point of taking a history?

Some would argue I am splitting hairs for

what is essentially a dimensional spectrum

of ’mood reactivity’. If so, I blame DSM-

IV (1) for inadequate guidance: ’Not

Otherwise Specified’ just does not

cut it for me.

I acknowledge that irritability and ex-

plosivity are very real symptoms, with roots

such as depressive or bipolar disorder,

substance intoxication, withdrawal or even

a tumour of the frontal lobe. But, perhaps

because of the plethora of attention given to

bipolar disorder, the public (and many

clinicians) now equates all anger-associated

behaviours to ’mood swings’.

What about other factors that might

distinguish, say, the anger of Antisocial

Personality features from the anger of the

supposedly rare Intermittent Explosive

Disorder – such as presence or absence of

remorse? Judging by responses to my

history taking, I wonder whether my

patients were ever asked about that, let

aloneotherDSM-IV (1) criteria.Whatdoes

that say about how rigorously these criteria

are being applied? Just using DSM-IV (1)

standards, plain depressive disorder or axis

II factors might ’better account for’ the

current phenomenon of anger spilling into

our emergency rooms and psychiatric

offices.

I now know that having ’anger’ in

America today means that you will end up

diagnosed with ’a disorder’. This invari-

ably leads to pharmacological rather than

psychologically based treatments – despite

all talk of ’combined approaches’. In

keeping with our insurance and pharma-

ceutically driven system, there is predict-

ably scant emphasis on the psychological

and interpersonal dimensions that char-

acterize most anger episodes and a dispro-

portionate emphasis on ’mood stabilizers’.

Maybe because axis II is not ’reimburs-

able’, clinicians’ use of it is actually dying –

along with all its psychological and psy-

chodynamic underpinnings. Add to this

the sheer brevity of 15-min ’med-checks’,

and now I understand why ’it’s not just

anger. it’s Bipolar II’.

Sumit Anand1,21Adolescents Diagnostic Service,

River Park Hospital,Huntington, West Virginia 25705,

and2Department of Psychiatry and Behavioral Medicine,

Marshall University,Huntington, West Virginia 25701,

USA.

Sumit AnandDepartment of Psychiatry and Behavioral Medicine,

Marshall University,Huntington, West Virginia 25701,

USA.Tel: 11 304 526 9111;Fax: 11 304 526 9301;

E-mail: drsumitanand@gmail.com

Acta Neuropsychiatrica 2007: 19:325

� 2007 The Author

Journal compilation � 2007 Blackwell

Munksgaard

DOI: 10.1111/j.1601-5215.2007.00221.x

Reference1. American Psychiatric Association.

Diagnostic and statisticalmanual ofmental

disorders, 4th edn. Washington, DC:

American Psychiatric Association, 1994.

325

Typical Aicardi syndrome in a male

Aicardi syndrome (AS), identified first by

Jean Aicardi in 1965 (1), is a rare genetic

disorder characterized by infantile spasm

(IS), corpus callosal agenesis and chorio-

retinal lacunae (CRL) (2). This is an X-

linked dominant condition and occurs

almost exclusively in females because of

early embryonic lethality in hemizygous

males (3). Because of its lethality, it has

been very rarely reported in boys (4–6),

with all patients having severe motor and

speech disabilities, intractable epilepsy and

a high mortality rate (3). Although mild

cases havebeen reported (7,8), this has been

exclusively in girls. We report the first case

of AS inmale withmildmental retardation

and normal speech development.

A 21-year-old male previously thought

to have infantile epilepsy was referred

because of increasing aggressive outbursts

and suspiciousness towards family

members. On evaluation of history, he

was found to have been born of non-

consanguineous marriage, of full term at

the hospital. The prenatal period was

eventful because of the presence of

eclampsia in the mother, diagnosed in the

second trimester (untreated), and he was

delivered by emergency lower segment

caesarean section because of prolonged

labour. His weight and head circumference

were normal. The Denver developmental

screening scale (9) revealed delayed devel-

opmental milestones. Around the age of

3–4 months, seizures manifested them-

selves for the first time, mainly of a focal

nature, characterised by clustering of both

shoulder shrugging and salaamattacks and

presence of status epilepticus.Till the ageof

20 years, these continued, although with

a decreased frequency, on treatment with

60 mg phenobarbitone. At the time of

evaluation, he was found to be mildly

intellectually impaired and with normal

speech. A complete medical examination

revealed right non-paralytic squint, spastic

left-sided hemiplegia and presence of

a wide forehead with thoracolumbar ky-

phoscoliosis.His facieswasnormal.Fundal

examination revealed choroidal calcifica-

tion, otherwise was entirely normal.

He was then investigated. Radiology

revealed no evidence of costovertebral

abnormality.

Interictal electroencephalography re-

vealed intermittent alpha and diffuse theta

activity in the background. Rhythmical

slowing (delta waves) with burst suppres-

sion was noted in the right side. Magnetic

resonance imaging revealed the presence

of complete callosal agenesis. An area of

dysplasia was noted in the right frontal

lobe with colpocephaly and convergence

of lateral ventricles. He was started on

800 mg carbamazepine along with 60 mg

phenobarbitone. Risperidone 3 mg was

added to manage his suspicion and

aggressive outbursts towards his family

members. On 1-year follow-up, patient

was maintaining well on the above med-

ications, with no recurrence of seizures or

aggressive outbursts.

Discussion

AS is a sporadic disorder that affects

primarily females and is hypothesised to

be caused byheterozygousmutations in an

X-linked gene. Its main features include

a triad of ISs, agenesis of the corpus

callosum and distinctive CRL. Additional

common findings include moderate to

profound mental retardation, grey matter

heterotopia, gyral anomalies and verte-

bral and rib defects. Most patients have

severe learning disability, intractable epi-

lepsy, agenesis of the corpus callosum and

reduced life expectancy (3).

Being a differential diagnosis of ISs, it

has been usually reported in infants and

children, with the exception of one case

(8). Our case is only the second such report

in adulthood and the first in a male,

showing that a milder form of AS exists

and that there may be a wide spectrum of

AS than has been described before (10–12).

He is only mildly mentally disabled, and

his epilepsy has been well controlled.

However, he does not have the typical

CRLpresent from childhood, but agenesis

of the corpus callosum along with other

biological markers of frontal dysplasia

and colpocephaly supports the diagnosis

of AS. The striking feature is that he has

adjusted well to his family till the age of

21 years, providing further evidence that

a mild form of the disease does exist. The

presenting complaint of increased aggres-

sion and suspicion in this patient could,

however, be linked to right hemispheric

dysfunction caused by epilepsy, hypothe-

sized to cause paranoid psychoses and

Capgras syndrome (13).

Even though complete agenesis has

been linked to severe mental retardation

(8), our case shows otherwise, suggesting

that there is no relation between the two.

His mental disability may be linked to

hormonal deficiency during neurogene-

sis, also seen in mental retardation

accompanying hypopituitarism (14).

Furthermore, most of these cases have

been described to have intractable epi-

lepsy; overall outlook need not necessar-

ily be gloomy keeping in view several

cases that have shown complete remission

(8,10) including ours. As efforts to map

a genetic locus have been unsuccessful so

far, an absolutely firm diagnosis will have

to await the discovery of a genetic

marker. Until such time, biological

markers may have to suffice, with hope

from functional magnetic resonance

imaging being able to further map out

dysfunctional cortical areas (15).

Sahoo Saddichha,Narayana Manjunatha,

Sayeed Akhtar

Central Institute of Psychiatry, Kanke, Ranchi,India, 834006.

Sahoo Saddichha,Central Institute of Psychiatry,

Kanke, Ranchi,834006, India.

Tel: 191 98353 69975;Fax: 191 65122 33668;

E-mail: saddichha@gmail.com

Acta Neuropsychiatrica 2007: 19:326–327

� 2007 The Authors

Journal compilation � 2007 Blackwell

Munksgaard

DOI: 10.1111/j.1601-5215.2007.00237.x

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COMMENT & CRITIQUE