Treatment-resistant mania in Dandy-Walker malformation with seizure disorder: A case report.

Dear Sir,

Dandy–Walker malformation (DWM) is a congenital malformation of the brain characterized by hypoplasia of cerebellar vermis, enlarged cistern magna, dilatation of the fourth ventricle, and enlarged posterior fossa.[12] Psychiatric co-morbidities such as mood disorders, obsessive compulsive disorder, schizophrenia, and attention deficit hyperkinetic disorders are reported with DWM.[13] The association of mood disorders, with DWM, is well documented, but co-morbid bipolar disorder is limited to case reports.

An 8-year-old boy with a diagnosis of DWM with seizure disorder and right hemiparesis was referred from a neurology unit for psychiatric consultation. Three months before, the patient had multiple episodes of generalized tonic-clonic seizure in a day. On the very next day, he had developed increased physical activity, over-talkativeness, inappropriate sexual behavior, social disinhibition, and decreased sleep. He had seizure disorder since the age of 3 years for which he was on irregular treatment. Past and family histories were insignificant. There was no developmental delay. Electroencephalogram revealed focal epileptiform discharges. Magnetic resonance imaging of brain had revealed enlarged fourth ventricle, hypoplastic vermis with corpus callosum agenesis along with dilatation of other ventricles and interhemispheric cyst [Figure 1].

Figure 1

Different sections of brain on magnetic resonance imaging (A) refer to hypoplastic vermis, (B) refer to dilated lateral ventricle, (C and D) refer to interhemispheric cyst, (E and F) refer to dilated cisterna magna, (G) refer to corpus callosum agenesis

The patient was hospitalized. On mental status examination, the patient was distractible. He had pressure of speech, elated mood, jocularity, plan for marriage, and hyper-sexuality. He was diagnosed with “mania without psychotic symptoms.” He was started with lithium 600 mg/day in divided doses, but developed tremor and ataxia, hence discontinued soon. Subsequently, he was treated with sodium valproate 500 mg/day and olanzapine 15 mg/day in divided doses, but due to poor response and weight gain, he was shifted to aripiprazole (15 mg/day) from olanzapine. However, the response was inadequate and psychomotor excitement persisted for which propranolol 20 mg/day was added, after which the patient had shown improvement.

Propranolol is effective in improving aggressive behavior, even in the treatment-resistant cases of mania.[2] Our patient had responded partially to pharmacotherapeutic intervention. Development of side effects to psychotropic drugs was due to underlying neurological abnormality.

Cerebellum has an important role in mood regulation; hence, malformation of the cerebellum has implication in bipolar disorder.[1] Our patient had a malformation of the cerebellum as well as agenesis of corpus callosum, dilated lateral ventricles, and large interhemispheric cyst, which are affecting the white matter integrity connecting frontal cortex; hence, may be responsible for manic symptoms, treatment resistance, and side effect proneness.

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