Cavernous sinus syndrome due Kalyan B. Bhattacharyya, Saurabh Rai 1 - - PDF document

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2006;56:243-45. 8. Duarri A, Teijido O, Lopez-Hernandez T, Scheper GC, Barriere H, Boor I, et al. Molecular pathogenesis of megalencephalic leukoencephalopathy with subcortical cysts: Mutations in MLC1 cause folding defects. Hum Mol Genet 2008;17:3728-39. Figure 2: Plain computed tomography scan of the brain showing symmetrical hypodensity in the white matuer and subcortjcal cysts in the temporal lobe

leading to coining of the name “Van der Knapp disease.” A Turkish study[3] later established that the disease had an autosomal recessive inheritance and Gorospe, Singhal and co‑workers performed a detailed genetic analysis and established the disease as a distinct entity with a common locus at the MLC 1 gene.[4] The disease is extremely rare in Eastern India and, to the best of our knowledge, only one

• ther case has been reported so far from West Bengal.[5]

Although common in children, there are few reports of adults with this syndrome.[5‑8] Canavan’s disease, Alexander’s disease, glutaric aciduria type 1 and Tay–Sach’s disease can also present with macrocephaly and symmetrical diffuse white matter edema in imaging, but the finding of subcortical cysts, particularly in the temporal lobes, strengthens the diagnosis of van der Knaap syndrome in this case.

Kalyan B. Bhattacharyya, Saurabh Rai1

Departments of Neurology, RG Kar Medical College, 1Bangur Institute of Neurosciences, Kolkata, West Bengal, India E-mail: kalyanbrb@gmail.com

References

1. Singhal BS, Gursahani RD, Biniwale AA, Udani VP. In: Proceedings

• f the 8th Asian and Oceanian Congress of Neurology, Tokyo, Japan:

Megaencephalic leukodystrophy in India; 1991. p. 72. 2. Cavalcanti CE, Nogueira A. Van Der Knaap syndrome. Megaencephaly with leucodystrophy. Report of 2 cases in the same family. Arq Neuropsiquiatr 2000;58:157-61. 3. Topçu M, Gartioux C, Ribierre F, Yalçinkaya C, Tokus E, Oztekin N, et al. Vacuoliting megaencephalic leucoencephalopathy with subcortical cysts, mapped to chromosome 22qtel. Am J Hum Genet 2000;66:733-9. 4. Gorospe JR, Singhal BS, Kainu T, Stephan WD, Trent J, Hoffman EP, et al. Indian Agarwal megaencephalic leucodystrophy with cysts is caused by a common MLC1 mutation. Neurology 2004;62:878-82. 5. Aditya S, Das Gupta R, Das D, Roy MK, Dhibar T, Das T. Van der Knaap syndrome: A case from West Bengal, India. Neurol Asia 2010;15:193-5. 6. Brockmann K, Finsterbusch J, Terwey B, Frahm J, Hanefeld F. Megalencephalic leukoencephalopathy with subcortical cysts in an adult: Quantitative proton MR spectroscopy and diffusion tensor MRI. Neuroradiology 2003;45:137-42. 7. Itoh N, Maeda M, Naito Y, Narita Y, Kuzuhara S. An adult case of megalencephalic leukoencephalopathy with subcortical cysts with S93L mutation in MLC1 gene: A case report and diffusion MRI. Eur Neurol Access this article online

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Cavernous sinus syndrome due to skull base metastasis: A rare presentation of hepatocellular carcinoma

Sir, We report the case of a 38‑year‑old man who presented with right‑sided headache, drooping of the eyelids, decreased right‑sided facial sensation and blurred right eye vision for 2 months. He also had abdominal distension, and loss of weight and appetite. On examination, he was emaciated and icteric, and had features of right‑sided cavernous sinus syndrome with right pupil dilated and sluggishly reacting to light, ptosis, impaired right eye extraocular movements, absent right‑sided facial sensation and atrophic right masseter. His abdomen revealed a hard nodular liver of 8 cm and ascites. An magnetic resonance imaging (MRI) of the brain revealed a large lobulated solid lesion involving the right cavernous sinus and encasing the cavernous part of the right internal carotid artery [Figure 1a and b] The computed

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Figure 1: Axial (a) and coronal T2-weighted image (b) showing a large T2 hypointense right cavernous sinus mass (black arrow) encasing the internal carotjd artery (ICA) (small white arrow), extending to the sphenoid sinus (white arrow) and masticator space (asterix). Axial post-contrast fat- suppressed image (c) shows moderate enhancement of the lesion (white arrow) and its extension to the posterior ethmoid sinuses and right orbital apex (black arrow). Also note the dural thickening and enhancement along the middle cranial fossa (arrowhead) a b c a b c Figure 2: Axial computed tomography (CT) image in the venous phase (a, b) shows an ill-defjned, hypodense right lobe lesion (white double arrows) against a background of chronic liver disease changes (black arrow) with ascites (white arrows); right portal vein thrombus (asterix) is also seen. Axial CT at the pelvic level (c) shows a permeatjve lesion with sofu tjssue in the right iliac bone (white arrow) suggestjve of metastasis

tomography (CT) scan revealed a tumor in the right lobe of the liver and in the portal vein with features characteristic

• f hepatocellular carcinoma (HCC) [Figure 2a‑c]. Ascitic

fluid analysis revealed low protein (0.8 g/dL), high serum ascites albumin gradient (2.6 g/dL) and total white blood cell count of 90 cells/cubic mm with 90% lymphocytes. Hepatitis B surface antigen was positive. Alfa fetoprotein (AFP) was 185,000 IU/mL. Biopsy of the lesion was deferred. The patient was initiated on symptomatic treatment. On explanation of the nature and prognosis of the disease, the patient’s family

• pted for palliative care.

The diagnosis of HCC in a patient with underlying chronic liver disease is established by the characteristic imaging features in MRI, along with elevated AFP levels. Biopsy is necessary only when the diagnostic imaging results are inconclusive. The most common sites of metastases are the lungs, lymph nodes and bones. Metastases to the skull is rare, found

• nly in 0.5–1.6% of patients.[1] Among these, the base of

skull was involved in 16 of 59 (27%) patients in a literature review.[2] Lee reported 16 cases of craniospinal metastases in HCC, of which six cases had skull metastases.[3] There is only one reported case of HCC presenting as cavernous sinus syndrome.[4] Palliative radiotherapy for skull metastases provides excellent pain relief and resolution of the cranial nerve deficits. Sorafenib, an oral multikinase inhibitor that blocks tumor cell proliferation and angiogenesis by acting on serine/threonine kinases Raf‑1/B‑Raf, tyrosine kinases of vascular endothelial growth factor receptor‑2/‑3 and platelet‑derived growth factor receptor ‑bis, is the only drug currently available for advanced

• HCC. The prognosis for patients with bone metastases remains

poor, with a median survival of just 7 months.

Ronald A. B. Carey, S. D. Nathaniel, Sohini Das, Sniya Sudhakar1

Departments of Medicine and 1Radiology, Christian Medical College, Vellore, Tamil Nadu, India E-mail: ronaldcarey@gmail.com

References

1. Chan CH, Trost N, McKelvie P, Rophael JA, Murphy MA. Unusual case of skull metastasis from hepatocellular carcinoma. ANZ J Surg 2004;74:710-3. 2. Guo X, Yin J, Jiang Y. Solitary skull metastasis as the fjrst symptom

• f hepatocellular carcinoma: Case report and literature review.

Neuropsychiatr Dis Treat 2014;10:681-6. 3. Lee JP. Hepatoma presenting as craniospinal metastasis: Analysis of sixteen cases. J Neurol Neurosurg Psychiatry 1992;55:1037-9. 4. Kao HJ, Cheng ST, Chen WH, Yin HL. Cavernous sinus syndrome and hepatoma metastasis. Kaohsiung J Med Sci 1998;14:117-20. [Downloaded free from http://www.neurologyindia.com on Friday, April 20, 2018, IP: 103.227.71.66]

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Access this article online

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“Fungimitosis”: Invasive fulminant Aspergillus brain infection mimicking gliomatosis cerebri

Sir, Fungi are commensals in humans.[1] However, whenever host defenses are at stake, fungi can lead to opportunistic infections, some of which can be lethal. Aspergillosis is an infection caused by fungi of the Aspergillus fumigatus family. Commonly, maxillary sinusitis of dental origin and pulmonary aspergillosis act as primary sources of the infection.[2] Central nervous system (CNS) aspergillosis can affect the cerebral parenchyma, the meninges or the vascular system. Rarely, the infection may also be air‑borne, contaminating the operative field during a neurosurgical procedure.[3] A 50‑year‑old male patient presented with gradually progressive, holocranial, and dull aching headache for 4 months, associated with memory deficits over a period of 3 months. He had mild clumsiness in using his right hand. There was neither any history

• f co‑morbid conditions nor any evident features suggestive of

an immunocompromised state. On examination, the patient had a mini‑mental state examination score of 24/30. His motor system examination was unremarkable except for positive pronator drift on the right side along with ipsilateral upgoing

• plantars. His hematological investigations were normal including

nonreactivity for human immunodeficiency virus (HIV). Cranial magnetic resonance imaging showed a diffuse lesion, hypointense on T1WI and hyperintense on T2WI, involving the left frontal, parietal and temporal lobes with patchy contrast enhancement. There was diffuse hemispheric edema of the same lobes with mass effect and midline shift [Figure 1a‑f]. These features were suggestive

• f gliomatosis cerebri. Hence, the patient underwent a left

fronto‑temporo‑parietal (FTP) craniotomy, left temporal lobectomy with decompression, and biopsy of the lesion from the frontal lobe via the middle frontal gyrus approach. At surgery, the lesion was found to be grayish white, minimally vascular, firm to hard (gritty) in consistency, which raised the suspicion of an infective etiology. Hence, microbiological staining (Gram’s, KOH preparation, acid‑fast bacillus staining) and cultures (aerobic, tubercular, and fungal) of the sample were sought, apart from its histopathogical examination. In the immediate postoperative period, the patient developed aphasia and right hemiplegia although this improved gradually over a period of 4 days. The histopathological examination showed fungal hyphae in the excised tissue and culture of the tissue suggested the diagnosis of aspergillus infection. The patient was started on intravenous amphotericin‑B

• n postoperative day 2 (dose 1.5 mg/kg wt/daily). During

the course in the hospital, the patient deteriorated in sensorium, and a repeat computed tomography (CT) of the brain showed diffuse cerebral edema with mass effect for which a left FTP decompressive craniectomy was

• performed. Over the next 2 days, the patient did not show

any improvement in the neurological status, prompting us to repeat his CT head. The CT scan showed dilated ventricles for which an external ventricular drainage was instituted. The patient, however, continued to deteriorate and finally succumbed to the illness on the 8th postoperative day. Invasive fungal infection involving the central nervous system (CNS) is often fulminant and fatal with a reported mortality of nearly 100% in the older literature. This disease was described as “almost always a clinical surprise” by Nadkarni and Goel.[4] These cases present an interesting diagnostic dilemma for the neurosurgeons and neuroradiologists, as neither the clinical nor the imaging features of CNS fungal infections are truly diagnostic of the disease and with the patient being immuno‑competent, the possibility of fungal infection is seldom considered in the list of clinical differentials.[5] Cerebral parenchymal lesions can range from fungal granuloma, diffuse cerebritis to frank abscess formation. A sinonasal disease with intracranial extension is the most frequent presentation although isolated parenchymal lesions without evidence

• f any sinonasal involvement are not infrequent. They

usually present as an heterogenous signal intensity lesion

• n T1WI and T2WI images, often showing areas of diffusion

restriction within. Because of rarity of the lesion and its non‑specific imaging findings, most cases are often confused with tumors and infarcts. In our case, involvement of one whole hemisphere mimicked gliomatosis cerebri; hence, we named it as “fungimitosis.” Impaired/poor nutritional status, injudicious use of steroids or antituberculous drugs, and parasitic infections are all considered as predisposing factors for fungal infections.[6,7]

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