Van der Knaap syndrome, a case from West Bengal, India

Leucodystrophies are a group of white matter diseases caused by an abnormality in the formation or maintenance of one or more components of the myelin sheath. The devastating course of the disease coupled with the lack of any defi nitive treatment render them potentially fatal and incurable. Van der Knaap Syndrome, also known as megaloencephalic leucoencephalopathy with subcortical cyst, is a relatively new and rare entity. Although reported sporadically from Europe, Asia and the Agarwal community in India, there is practically no case report from the eastern part of India. We present a case of van der Knaap syndrome in an adult female with some atypical features.

Rosai-Dorfman disease.

We describe two cases of Rosai-Dorfman disease. One of them had commonly described cervical adenopathy and the second with the very rare bilateral orbital involvement. Both our cases required treatment with steroids because of the danger of pressure symptoms and disfigurement and multiple episodes of high fever in one of them.

Limbic encephalitis--an uncommon presentation of systemic malignancy.

A 46 years, nondiabetic, nonhypertensive woman presented with headache, vomiting, low grade intermittent fever, behavioral abnormality and seizures for last three months. Clinically she had meningism with bilateral papilloedema. Based on CSF analysis, normal CT scan of brain and suspicious lesion in X-ray chest, she was put on anti-tuberculosis therapy. As the patient further deteriorated clinically, MRI of brain was carried out and revealed bilateral increased signal intensities in both medial temporal lobes in T2 and flair sequences. Inj. acyclovir was added considering the diagnosis of herpes simplex encephalitis. In spite of receiving treatment patient gradually became more drowsy and repeat X-ray chest with CT guided FNAC showed picture of adenocarcinoma of lung. So finally, we concluded it to be a case of limbic encephalitis.

Bilateral intracerebral haemorrhages: an atypical presentation of Japanese encephalitis.

Japanese encephalitis is common human endemic encephalitis seen over various parts of the world. Usual presenting features include an encephalitic syndrome, symptoms of frontal lobe, basal ganglia and thalamic involvement. Characteristic radiological picture is bilateral thalamic and basal ganglia hypo density in the CT scan and hypo-intensity in T1 and hyperintensity in T2 weighted image in MRI. Very rarely occurrence of bilateral hemorrhage may be seen in these regions. This radiological change may be early indicator of the disease before serological confirmation by the available diagnostic modalities. In this communication, we have reported a case of Japanese encephalitis presented with bilateral basal ganglia hemorrhages.

Acute intracerebral haematoma--an unusual presentation of herpes simplex encephalitis.

A young man presented with acute headache, behavioral abnormality and CT scan evidence of intracerebral hematoma. Detailed investigation established its infective origin due to herpes simplex virus type-I. The patient made an excellent recovery following a course of acyclovir. Frank intracerebral haematoma in the form of well defined mass on CT scan in patient with herpes simplex encephalitis is extremely rare.

Compressive myelopathy due to nocardiosis from dermal lesion.

Nocardiosis refers to locally invasive or disseminated infection associated with Nocardia species. Most infections enter through respiratory tract and then disseminate systemically. Rarely primary nocardial infection of the skin of the back may spread to contiguous structures including vertebrae. A 30 years male presented with indolent skin lesion in the upper dorsal region of the back for one year following an accident and subsequently developed features of spinal cord compression and parenchymal involvement of lung. The rarity of such type of spread in an immuno-competent individual has been highlighted.