Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 54, Num. 4, 2006, pp. 450-451

Neurology India, Vol. 54, No. 4, October-December, 2006, pp. 450-451

Letter To Editor

Adult onset hemiparkinsonism with brain hemihypoplasia

Matsui Hideaki, Nishinaka Kazuto, Oda Masaya, Komatsu Kenichi, kubori Tamotsu, Udaka Fukashi

Department of Neurology, Sumitomo Hospital, 5-3-20 Nakanoshima, Kita-ku, Osaka, 530-0005
Correspondence Address:Department of Neurology, Sumitomo Hospital, 5-3-20 Nakanoshima, Kita-ku, Osaka, 530-0005, matsui-hideaki@sumitomo-hp.or.jp

Code Number: ni06160

Sir,

A 47-year-old, right-handed man was referred to our department for postural and rest tremor in the left arm and severe clumsiness in the left upper and lower limbs that occurred at age 45. These symptoms gradually deteriorated over the first year, then disease progression halted at Hoehn-Yahr I stage. He was prescribed 300mg levodopa by another hospital for six months, but this had no beneficial effects.

Family and personal history were unremarkable. The patient was born after an uncomplicated pregnancy and delivery. The patient grew well, but did not perform well in any kinds of sports in his youth.

Neurological examination disclosed left postural and rest tremor with lead-pipe-like rigidity and severe bradykinesia/clumsiness in ipsilateral upper and lower limbs. Tremor was postural-dominant. The right limbs showed completely normal findings and absolutely normal intellectual performance was demonstrated. There were no abnormalities in sensory, autonomic or cerebellar systems. Deep tendon reflex was normal in the right limbs and was exaggerated in the left. Pathological reflexes were not seen. No clinical evidence of hemiatrophy was seen.

General blood examination including thyroid function, electrocardiogram and chest X-rays were within normal limits. Nerve conduction, sensory evoked potentials and magnetic resonance imaging (MRI) of the cervical cord did not disclose any abnormal findings. Cardiac Metaiodobenzylguanidine (MIBG) uptake was normal[Figure - 1].

Brain MRI showed right brain hemihypoplasia [Figure - 2]. N-isopropyl-p- ¹²³sub I iodoamphetamine ( ¹²³ I-IMP) single photon emission computed tomography (SPECT) showed right cerebral hypoperfusion [Figure - 3].

The patient was diagnosed with hemiparkinsonism and brain hemihypoplasia and symptoms did not change since diagnosis.
In our patient, diagnosis of secondary parkinsonism rather than idiopathic Parkinson's disease was justified by normal MIBG uptake (decreased cardiac MIBG uptake was reported in idiopathic Parkinson's disease) and the lack of drug sensitivity.
Brain hemihypoplasia is a rare condition that has been rarely observed in association with parkinsonism, but is generally related to epilepsy, congenital encephalopathies and intellectual retardation.[1] The etiology of brain hypoplasia remains unclear.
There appear to be few reports on hemiparkinsonism with brain hemihypoplasia. Giladi, et al reported 11 patients with hemiparkinsonism-hemiatrophy syndrome and in which four patients were described as having contralateral cortical atrophy.[2] In some cases, contralateral substantia nigra abnormality was demonstrated.[2],[3] Among the reported cases, the patient in Marchionii's case report was quite similar to ours.[4] Magnetic resonance imaging and clinical findings were almost the same, including left-side symptoms and right-side brain hemihypoplasia. That patient showed skeletal left-side hemiatrophy; but in our case, we could not perform Xp examination of the foot and hand because of patient's refusal.

In general, symptoms due to hemihypoplasia do not progress with time. In our patient, disease progression has, at least at present, stopped; however, major symptoms were adult onset-related. Reasons why disease symptoms are not congenital and they only appear at the adult stage, are unknown.

In conclusion, we report a case of hemiparkinsonism due to brain hemihypoplasia. Hemihypoplasia should be considered as one of the causes of adult onset hemiparkinsonism.

References

Copyright 2006 - Neurology India

The following images related to this document are available:

Photo images