Subacute Sclerosing Panencephalitis Presenting as Mania

To the Editor: Subacute sclerosing panencephalitis (SSPE) is a rare, invariably fatal degenerative disease of the CNS developing after measles infection. Besides neurological problems as initial presenting symptoms, rare reports of its presentation with pure psychiatric symptoms have been reported. The authors here report a case of 14-year-old boy who initially presented with manic symptoms and was subsequently diagnosed to be suffering from SSPE. Importance of ruling our organic conditions is emphasized.

Subacute sclerosing panencephalitis (SSPE) is a rare, invariably fatal degenerative disease of the central nervous system that afflicts mainly children and adolescents. Although for some time now it has been known to develop after measles infection, the exact mechanism of its causation is still not fully understood. Initial presenting symptoms have commonly been reported to be neurological-like myoclonic jerks, falling attacks, changing gait, abnormal movements, speech impairment, inability to walk or stand, seizures, dementia, visual disturbances, and pyramidal and extrapyramidal signs. Although behavioral disturbances have been reported to be the initial presentation in some cases, reports of cases with purely psychiatric symptoms as the initial complaints are rare.¹

We hereby report a case of 14-year-old boy who was initially diagnosed with a manic episode and who subsequently developed myoclonic jerks. EEG and CSF findings confirmed a diagnosis of SSPE.

Case Report

A 14-year-old boy working as a helper in a shop, was brought to us by his mother for complaints of behavioral change over the past 1 month. She reported that he had not been working properly. He would demand money and would go out frequently to watch movies. He would remain outside the home most of the time and talked a lot. He would look more cheerful then before, would not obey family members, and became irritable and angry. His hygiene and grooming also were increased, and he would take three or four baths per day. At home, he would pace here and there. His sleep was markedly decreased, from 7–8 hours before to 3–4 hours, and he did not report any fatigue. There was no past or family history of any psychiatric illness. Mental status examination revealed a restless boy, with increased psychomotor activity, with eye contact established but not maintained. His speech was increased in pitch, tone, and volume. His affect was elated. Flow of thought was increased and revealed ideas of grandiosity. He had no insight into the illness. His higher mental functions were, however, normal. Detailed neurological examination including fundus examination was normal. We made a diagnosis of first-episode mania. Routine hematological investigations including chest X-ray and ECG were normal. He was started on olanzapine 5 mg per day, increased to 10 mg after 5 days.

The patient was followed up after about 10 days, and his mother reported that he was not able to tolerate the medication. He was unduly sedated and would at times pass urine in clothes. Also, now she reported that the patient would have repeated spontaneous jerky movements for a very short period of time. On examination at this time, the patient had myoclonic jerks, and his gait was unsteady. His affect was inappropriate. Olanzapine was stopped. He was further investigated. MRI of the brain revealed multiple areas of hyper intensities in bilateral brain parenchyma on T2-weighted images that were more in the posterior part of cortex. The patient was subsequently referred to a neurologist. Blood tests for toxoplasma, cytomegalovirus, and herpes simplex virus IgG and IgM were negative. Venereal disease research laboratories (VDRL) slide test was negative. EEG revealed a typical picture of generalized periodic spike and wave formation, with periodicity at every 7 sec. CSF was grossly clear, with normal opening pressure and showed 5 lymphocytes/cm³, protein 20 mg/dl, and sugar 70 mg/dl against a blood glucose of 100 mg/dl. There were elevated anti-measles IgG and IgM anti bodies. A diagnosis of SSPE was made. His condition deteriorated, with increase in frequency of myoclonic jerks with urinary and fecal incontinence over the next 4 weeks. He could not take care of himself and had to be assisted by family members for his daily routine. His gait became progressively unsteady, with frequent falls, as well. He was started on sodium valproate 400 mg per day along with clonazepam 1 mg per day. The patient was subsequently lost to follow-up.

Discussion

Our patient fulfilled the criteria for SSPE, which include fulfillment of at least three of the five following criteria:²

Presentations with psychiatric symptoms have been described in the literature. Cases of schizophreniform psychosis, paranoid psychosis, schizophrenia, catatonia, and depression are well described.^3–7 However, to the best of our knowledge, case presentation with typical features of mania has not been reported, although aimless wandering, hyperreligiosity, irritability, and adamant behavior have been described as presenting feature in a few cases.⁶

Our case highlights the importance of ruling out organic conditions, especially in adolescents without any known risk factors for mania. Also, it highlights the importance of EEG, which can be an inexpensive mode of investigation, in patients with psychiatric symptoms.

In conclusion, SSPE can present initially with purely psychiatric symptoms, and pediatricians, neurologists, as well as psychiatrists, should be aware of this rare possibility. A high index of suspicion is needed to detect SSPE in its atypical and rare forms. When faced with a young patient with psychiatric manifestations, with no other significant history, SSPE is always a diagnosis to be excluded.