Letters to Editor Internal jugular, subclavian and brachiocephalic vein thrombosis associated with cerebral venous sinus thrombosis Sir, A 23‑year‑old lady presented on day‑12 of postpartum with 3 days history of headache and two episodes of generalized tonic‑clonic seizures. Examination revealed bilateral papilledema and mild left‑sided hemiparesis. Magnetic resonance imaging and venography showed [Figure 1] extensive thrombosis of superior sagital, bilateral lateral, and straight sinus with right thalamic hyperintensities. She was treated with anticoagulation and anti epileptics and supportive measures. Investigations showed low hemoglobin and hyperhomocysteinemia as risk factors apart from puerperal state. On day‑10 of admission, she developed left‑sided neck pain and swelling of left upper limb. Computerized tomographic venography [Figure 2] showed thrombosis of left internal jugular (IJV),
subclavian (SV), and brachiocephalic veins (BV). She improved with optimization of anticoagulation treatment over the next 10 days. Though there are few reports of IJV thrombosis with cerebral venous thrombosis[1,2]; however, involvement of simultaneous three veins (IJV, SV, and BV) has not been reported earlier as per our knowledge.
Girish Baburao Kulkarni, Veerendrakumar Mustare, Vinod Varghese Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
References
1. Beer‑Furlan A, de Almeida CC, Noleto G, Paiva W, Ferreira AA, Teixeira MJ. Dural sinus and internal jugular vein thrombosis complicating a blunt head injury in a pediatric patient. Childs Nerv Syst 2013;29:1231‑4. 2. Mittal S, Garg P, Verma S, Bhoriwal S, Aggarwal S. Internal jugular vein thrombosis: An uncommon presentation. Vascular 2013;21:267-9. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.121935
Received: 21-08-2013 Review completed: 06-09-2013 Accepted: 13-10-2013 a
c
b
Figure 1: Magnetic resonance imaging of brain T1 axial section (a), T2 sagital section (b) and magnetic resonance venography (c) showing thrombosis of superior sagital bilateral lateral sinus and straight sinus
a
c
b
d
Figure 2: Computerised tomographic venography coronal (a,b) and axial (c,d) showing thrombosis of internal jugular, subclavian, and brachiocephalic veins on left side
526
Cerebral pheohyphomycosis: Report of a rare case with review of literature Sir, Pheohyphomycosis is a group of fungal infections characterized by presence of septate pigmented hyphae in the tissues and includes cutaneous, subcutaneous and systemic infections. [1] Central nervous system pheohyphomycosis is very rare and presents with unusual features and is associated with poor prognosis without appropriate treatment.[2] A 65‑year‑old male patient presented with fever and later developed left sided hemiparesis of 1 month duration. Contrast computed tomography (CT) and magnetic resonance imaging showed a ring enhancing lesion in the right frontal lobe [Figure 1]. Excision of Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Letters to Editor
the mass was performed. Gross examination showed multiple gray white soft‑tissue fragments measuring 1 cc in aggregate. Microscopic examination showed portions of reactive glial tissue with central area of necrosis and dense infiltrate of neutrophils, lymphocytes and plasma cells with scattered foreign body type of giant cells and fungal hyphae with pigmented walls. There were also focal micro abscesses composed of septate, thin mycelial filaments and spores with brown pigmentation surrounded by chronic inflammatory infiltrates [Figures 2 and 3]. Periodic acid Schiff stain showed PAS positive fungal hyphae‑slender, septate hyphae with conidia and spores [Figure 4a]. The fungal elements were also positive on Masson Fontana staining indicating the presence of melanin [Figure 4b]. A final impression of cerebral pheohyphomycosis was given. The postoperative course was uneventful, itraconazole was continued and he was discharged after 2 weeks. He is doing well and attends the outpatient department regularly.
(1) cutaneous (2) subcutaneous (3) paranasal sinus and (4) cerebral types.[1] Cerebral pheohyphomycosis is a very rare, but the most serious form of disease, usually caused by Cladophialophora bantiana.[2] Less common causes include Ramichloridium mackenzei, Ochroconis gallopavum, Thielavia subthermophila and Fonsecaea species.[3] The portal of entry is not known, though inhalation of spores into the lung and colonization and subsequent hematogenous spread has been suggested.[2] Life‑threatening fungal infections are often associated with severe immunocompromised states. However, primary cerebral pheohyphomycosis appears to be an exception to this rule, since it is increasingly recognized as a cause of serious disease in patients with or without immunodeficiency and patients are often immunocompetent with no known underlying diseases.[2] Presence of melanin in these fungi provides a protective advantage in evading host defenses and may be responsible for its pathogenic potential.[4]
Pheohyphomycosis is the term used for infections caused by dematiaceous fungi with characteristic presence of melanin pigment in their cell walls. Clinically, phaeohyphomycosis has been classified according to the anatomical site involved as
a
b
Figure 1: (a) Computed tomography and (b) magnetic resonance imaging showing a ring enhancing lesion in the right frontal lobe
a
b Figure 3: (a) Foreign body giant cells and pigmented fungal hyphae (H and E, ×100) (b) foreign body giant cells containing pigmented fungal hyphae (H and E, ×200)
Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Figure 2: Reactive glial tissue with scattered foreign body giant cells and inflammatory cells (H and E, ×40)
a
b Figure 4: (a) Fungal hyphae, conidia and spores positive for periodic acid Schiff (×200) (b) fungal elements showing positivity on Masson Fontana staining (×200)
527
Letters to Editor
Symptoms and signs are largely nonspecific mostly as a result of pressure effect leading to headache, papilledema and hemiparesis. It clinically presents usually as brain abscess, rarely as chronic meningitis, encephalitis, myelitis or significant cerebrospinal fluid eosinophilia and very rarely as an intraventricular mass. [5] CT typically show an irregular variably contrast enhancing mass with surrounding hypodense edema, mimicking high grade gliomas or metastatic lesions in brain.[6] On histopathological examination, they usually have a characteristic appearance of irregularly swollen hyphae with yeast‑like structures. Masson‑Fontana stain for melanin is used to confirm the presence of dematiaceous hyphae. Prognosis is very poor without treatment. There is no standard therapy for these often fatal infections, and few studies reported that a combination of amphotericin B, 5‑fluorouracil and itraconazole was associated with improved survival.[7]
Shantha Ravisankar, R. Vimal Chander1 Department of Pathology, Institute of Neurology, Madras Medical College, 1Department of Pathology, Saveetha Medical College, Thandalam, Chennai, Tamil Nadu, India E‑mail: [email protected]
References 1. 2. 3.
4. 5. 6. 7.
Kumar KK, Hallikeri K. Phaeohyphomycosis. Indian J Pathol Microbiol 2008;51:556‑8. Revankar SG, Sutton DA, Rinaldi MG. Primary central nervous system phaeohyphomycosis: A review of 101 cases. Clin Infect Dis 2004;38:206‑16. Sutton DA, Slifkin M, Yakulis R, Rinaldi MG. U.S. case report of cerebral phaeohyphomycosis caused by Ramichloridium obovoideum (R. mackenziei): Criteria for identification, therapy, and review of other known dematiaceous neurotropic taxa. J Clin Microbiol 1998;36:708‑15. Revankar SG, Patterson JE, Sutton DA, Pullen R, Rinaldi MG. Disseminated phaeohyphomycosis: Review of an emerging mycosis. Clin Infect Dis 2002;34:467‑76. Sujit Kumar GS, Dugar M, Chacko G. Cerebral phaeohyphomycosis presenting as an intraventricular mass. Neurol India 2006;54:102‑3. Hauck EF, McGinnis M, Nauta HJ. Cerebral phaeohyphomycosis mimics high‑grade astrocytoma. J Clin Neurosci 2008;15:1061‑6. Sharkey PK, Graybill JR, Rinaldi MG, Stevens DA, Tucker RM, Peterie JD, et al. Itraconazole treatment of phaeohyphomycosis. J Am Acad Dermatol 1990;23:577‑86.
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.121936
Received: 18‑05‑2013 Review completed: 29‑07‑2013 Accepted: 20‑10‑2013 528
Magnetic resonance imaging features in seizures associated with nonketotic hyperglycemia Sir, A 57‑year‑old female was admitted for episodic clonic jerks affecting her right face and arm, each episode lasting for approximately 2′ of 10 days duration. There was no improvement of jerks with carbamazepine, instead there was increase in the frequency (every 5′) and also developed right upper limb weakness. Past medical history was negative. Neurological examination revealed right hemiparesia. Admission serum glucose was 29.8 mmol/L with no ketone bodies in the urine; serum sodium was 133.8 mmol/L and potassium was 3.5 mmol/L; blood urea nitrogen was 6.0 mmol/L and calculated serum osmolality was 310.4 mmol/L. Admission computed tomography was normal. Electroencephalography (EEG) revealed inter‑ictal epileptiform discharges around the left central sulcus [Figure 1a‑c]. Brain magnetic resonance imaging (MRI) [Figure 2] done on day 2 of admission showed subcortical hypointensity signal changes in the left parietal region on T2-weighted (T2‑W), fluid attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI). Apparent diffusion coefficient (ADC) map showed isointensity in the corresponding region. Overlying cortical showed hyperintensity on FLAIR, DWI sequences and hypointensity on ADC map. The ADC values in cortical and subcortical lesions were lower than contralateral normal regions, especially in cortical lesions. T1‑W sequences were normal. She was diagnosed as epileptia partialis continua (EPC) associated with nonketotic hyperglycemia (NKH). Carbamazepine was stopped and intravenous insulin therapy was started. She had good glycemic control and the jerking remitted completely within 72 h. Follow up MRI at 5 months showed complete resolution of cortical hyperintensity and subcortical hypointensity. Epileptic seizures are common in hyperglycemia and are often presenting features, particularly in NKH with blood glucose levels more than 20 mmol/L. [1‑3] NKH‑related seizures are mostly focal motor, sometimes it can be EPC. In this patient, hyperglycemia was de novo. In patients presenting with EPC, NKH should be considered and ruled out. Seizures associated with NKH are resistant to antiepileptic drugs and remit with correction of hyperglycemic state. [4] The precise pathgenesis of EPC in patients with NKH Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
subclavian (SV), and brachiocephalic veins (BV). She improved with optimization of anticoagulation treatment over the next 10 days. Though there are few reports of IJV thrombosis with cerebral venous thrombosis[1,2]; however, involvement of simultaneous three veins (IJV, SV, and BV) has not been reported earlier as per our knowledge.
Girish Baburao Kulkarni, Veerendrakumar Mustare, Vinod Varghese Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
References
1. Beer‑Furlan A, de Almeida CC, Noleto G, Paiva W, Ferreira AA, Teixeira MJ. Dural sinus and internal jugular vein thrombosis complicating a blunt head injury in a pediatric patient. Childs Nerv Syst 2013;29:1231‑4. 2. Mittal S, Garg P, Verma S, Bhoriwal S, Aggarwal S. Internal jugular vein thrombosis: An uncommon presentation. Vascular 2013;21:267-9. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.121935
Received: 21-08-2013 Review completed: 06-09-2013 Accepted: 13-10-2013 a
c
b
Figure 1: Magnetic resonance imaging of brain T1 axial section (a), T2 sagital section (b) and magnetic resonance venography (c) showing thrombosis of superior sagital bilateral lateral sinus and straight sinus
a
c
b
d
Figure 2: Computerised tomographic venography coronal (a,b) and axial (c,d) showing thrombosis of internal jugular, subclavian, and brachiocephalic veins on left side
526
Cerebral pheohyphomycosis: Report of a rare case with review of literature Sir, Pheohyphomycosis is a group of fungal infections characterized by presence of septate pigmented hyphae in the tissues and includes cutaneous, subcutaneous and systemic infections. [1] Central nervous system pheohyphomycosis is very rare and presents with unusual features and is associated with poor prognosis without appropriate treatment.[2] A 65‑year‑old male patient presented with fever and later developed left sided hemiparesis of 1 month duration. Contrast computed tomography (CT) and magnetic resonance imaging showed a ring enhancing lesion in the right frontal lobe [Figure 1]. Excision of Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Letters to Editor
the mass was performed. Gross examination showed multiple gray white soft‑tissue fragments measuring 1 cc in aggregate. Microscopic examination showed portions of reactive glial tissue with central area of necrosis and dense infiltrate of neutrophils, lymphocytes and plasma cells with scattered foreign body type of giant cells and fungal hyphae with pigmented walls. There were also focal micro abscesses composed of septate, thin mycelial filaments and spores with brown pigmentation surrounded by chronic inflammatory infiltrates [Figures 2 and 3]. Periodic acid Schiff stain showed PAS positive fungal hyphae‑slender, septate hyphae with conidia and spores [Figure 4a]. The fungal elements were also positive on Masson Fontana staining indicating the presence of melanin [Figure 4b]. A final impression of cerebral pheohyphomycosis was given. The postoperative course was uneventful, itraconazole was continued and he was discharged after 2 weeks. He is doing well and attends the outpatient department regularly.
(1) cutaneous (2) subcutaneous (3) paranasal sinus and (4) cerebral types.[1] Cerebral pheohyphomycosis is a very rare, but the most serious form of disease, usually caused by Cladophialophora bantiana.[2] Less common causes include Ramichloridium mackenzei, Ochroconis gallopavum, Thielavia subthermophila and Fonsecaea species.[3] The portal of entry is not known, though inhalation of spores into the lung and colonization and subsequent hematogenous spread has been suggested.[2] Life‑threatening fungal infections are often associated with severe immunocompromised states. However, primary cerebral pheohyphomycosis appears to be an exception to this rule, since it is increasingly recognized as a cause of serious disease in patients with or without immunodeficiency and patients are often immunocompetent with no known underlying diseases.[2] Presence of melanin in these fungi provides a protective advantage in evading host defenses and may be responsible for its pathogenic potential.[4]
Pheohyphomycosis is the term used for infections caused by dematiaceous fungi with characteristic presence of melanin pigment in their cell walls. Clinically, phaeohyphomycosis has been classified according to the anatomical site involved as
a
b
Figure 1: (a) Computed tomography and (b) magnetic resonance imaging showing a ring enhancing lesion in the right frontal lobe
a
b Figure 3: (a) Foreign body giant cells and pigmented fungal hyphae (H and E, ×100) (b) foreign body giant cells containing pigmented fungal hyphae (H and E, ×200)
Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Figure 2: Reactive glial tissue with scattered foreign body giant cells and inflammatory cells (H and E, ×40)
a
b Figure 4: (a) Fungal hyphae, conidia and spores positive for periodic acid Schiff (×200) (b) fungal elements showing positivity on Masson Fontana staining (×200)
527
Letters to Editor
Symptoms and signs are largely nonspecific mostly as a result of pressure effect leading to headache, papilledema and hemiparesis. It clinically presents usually as brain abscess, rarely as chronic meningitis, encephalitis, myelitis or significant cerebrospinal fluid eosinophilia and very rarely as an intraventricular mass. [5] CT typically show an irregular variably contrast enhancing mass with surrounding hypodense edema, mimicking high grade gliomas or metastatic lesions in brain.[6] On histopathological examination, they usually have a characteristic appearance of irregularly swollen hyphae with yeast‑like structures. Masson‑Fontana stain for melanin is used to confirm the presence of dematiaceous hyphae. Prognosis is very poor without treatment. There is no standard therapy for these often fatal infections, and few studies reported that a combination of amphotericin B, 5‑fluorouracil and itraconazole was associated with improved survival.[7]
Shantha Ravisankar, R. Vimal Chander1 Department of Pathology, Institute of Neurology, Madras Medical College, 1Department of Pathology, Saveetha Medical College, Thandalam, Chennai, Tamil Nadu, India E‑mail: [email protected]
References 1. 2. 3.
4. 5. 6. 7.
Kumar KK, Hallikeri K. Phaeohyphomycosis. Indian J Pathol Microbiol 2008;51:556‑8. Revankar SG, Sutton DA, Rinaldi MG. Primary central nervous system phaeohyphomycosis: A review of 101 cases. Clin Infect Dis 2004;38:206‑16. Sutton DA, Slifkin M, Yakulis R, Rinaldi MG. U.S. case report of cerebral phaeohyphomycosis caused by Ramichloridium obovoideum (R. mackenziei): Criteria for identification, therapy, and review of other known dematiaceous neurotropic taxa. J Clin Microbiol 1998;36:708‑15. Revankar SG, Patterson JE, Sutton DA, Pullen R, Rinaldi MG. Disseminated phaeohyphomycosis: Review of an emerging mycosis. Clin Infect Dis 2002;34:467‑76. Sujit Kumar GS, Dugar M, Chacko G. Cerebral phaeohyphomycosis presenting as an intraventricular mass. Neurol India 2006;54:102‑3. Hauck EF, McGinnis M, Nauta HJ. Cerebral phaeohyphomycosis mimics high‑grade astrocytoma. J Clin Neurosci 2008;15:1061‑6. Sharkey PK, Graybill JR, Rinaldi MG, Stevens DA, Tucker RM, Peterie JD, et al. Itraconazole treatment of phaeohyphomycosis. J Am Acad Dermatol 1990;23:577‑86.
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.121936
Received: 18‑05‑2013 Review completed: 29‑07‑2013 Accepted: 20‑10‑2013 528
Magnetic resonance imaging features in seizures associated with nonketotic hyperglycemia Sir, A 57‑year‑old female was admitted for episodic clonic jerks affecting her right face and arm, each episode lasting for approximately 2′ of 10 days duration. There was no improvement of jerks with carbamazepine, instead there was increase in the frequency (every 5′) and also developed right upper limb weakness. Past medical history was negative. Neurological examination revealed right hemiparesia. Admission serum glucose was 29.8 mmol/L with no ketone bodies in the urine; serum sodium was 133.8 mmol/L and potassium was 3.5 mmol/L; blood urea nitrogen was 6.0 mmol/L and calculated serum osmolality was 310.4 mmol/L. Admission computed tomography was normal. Electroencephalography (EEG) revealed inter‑ictal epileptiform discharges around the left central sulcus [Figure 1a‑c]. Brain magnetic resonance imaging (MRI) [Figure 2] done on day 2 of admission showed subcortical hypointensity signal changes in the left parietal region on T2-weighted (T2‑W), fluid attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI). Apparent diffusion coefficient (ADC) map showed isointensity in the corresponding region. Overlying cortical showed hyperintensity on FLAIR, DWI sequences and hypointensity on ADC map. The ADC values in cortical and subcortical lesions were lower than contralateral normal regions, especially in cortical lesions. T1‑W sequences were normal. She was diagnosed as epileptia partialis continua (EPC) associated with nonketotic hyperglycemia (NKH). Carbamazepine was stopped and intravenous insulin therapy was started. She had good glycemic control and the jerking remitted completely within 72 h. Follow up MRI at 5 months showed complete resolution of cortical hyperintensity and subcortical hypointensity. Epileptic seizures are common in hyperglycemia and are often presenting features, particularly in NKH with blood glucose levels more than 20 mmol/L. [1‑3] NKH‑related seizures are mostly focal motor, sometimes it can be EPC. In this patient, hyperglycemia was de novo. In patients presenting with EPC, NKH should be considered and ruled out. Seizures associated with NKH are resistant to antiepileptic drugs and remit with correction of hyperglycemic state. [4] The precise pathgenesis of EPC in patients with NKH Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
