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Good’s syndrome (thymoma with immunodeficiency): An unusual clinical presentation of bulbar symptoms and peripheral neuropathy Article · September 2012 CITATIONS

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5 authors, including: Nevein Botross

Foong kee Kan

Monash University (Malaysia)

Gleneagles Medini Hospital

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Amgad Riad

Rusli Bin Nordin

Newcastle University Medicine Malaysia

Monash University (Malaysia)



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Scottish Journal of Arts, Social Sciences and Scientific Studies - ISSN 2047-1278 http://scottishjournal.co.uk

SCOTTISH JOURNAL OF

ARTS, SOCIAL SCIENCES AND

SCIENTIFIC STUDIES

VOLUME 4, ISSUE I SEPTEMBER, 2012

Articles Effects of Pore-Fluid on Attenuation of Seismic Waves through Sedimentary Rocks from Southwestern Nigeria. 3 Olorode, Deborah Oluwaseun

3

Olatinsu, Olawale Babatunde

3

Odeyemi, Samuel Olusegun

3

Good’s syndrome (thymoma with immunodeficiency): An unusual clinical presentation of bulbar symptoms and peripheral neuropathy 12 Nevein PB

12

Kan FK

12

Amgad AR

12

Shanthi V

12

Rusli BN

12

Idealist Conception of Education: Plato’s Educational Ideas and its Relevance to Modern Day Education in Nigeria 22 Akinkuotu Yemi Ambrose (Ph.D.)

22

Ajisegiri Lookman Olaseni (B.A., P.G.D.E.)

22

Determining the Effect of Guessing on Test Scores: An Empirical Analysis

32

William J. Ubulom, PhD

32

Clifford M. Amini, PhD

32 1


Maxwell D. Eremie, EdD

32

G. Solomon Osho, PhD

32

Don Anthony Woods, PhD

32

Psychological and Social Gratification Factors Related to Pathological Gaming among Undergraduate Students 40 Ng You Ming

40

Bahaman Abu Samah

40

Steven Eric Krauss

40

Turiman Suandi

40

The Impact of Recreation Therapy Intervention on Festive Stress; Evidence from Nigeria 56 Busari, A.O. Ph.D

56

The Relationship of Employee Benefits and Career Development on Employee Resistance to Change 70 Ghazi H. Al Oufi

70

Abdul-Halim

70

Abdul-Majid

70

Husna Johari

70

Vegetation Characteristics of Lekki Peninsula of Lagos, Nigeria

83

Olatunde Ogunkunle

Environmental Conservation and the Implications for Contemporary Society

83

Development

of

Cultural

Tourism: 92

Ojua, Takim Asu (Phd)

92

Tangban, Egbe Ebagu

92

Abia Raphael Pius (Phd)

92

Bisong, Patrick Owan

92

2


Effects of Pore-Fluid on Attenuation of Seismic Waves through Sedimentary Rocks from Southwestern Nigeria. Olorode, Deborah Oluwaseun Department of Physics University of Lagos, Akoka. Lagos. Nigeria [email protected]; [email protected]

Olatinsu, Olawale Babatunde Department of Physics, University of Lagos, Akoka. Lagos. Nigeria [email protected]

Odeyemi, Samuel Olusegun Department of Physics,University of Lagos, Akoka. Lagos. Nigeria

Abstract Laboratory attenuation measurements using pulse transmission coupled with spectral amplitude wave-ratio technique were carried out on three sedimentary rocks namely: shale, sandstone and glauconite from Ewekoro in Southwestern Nigeria. Attenuation coefficient (k) was measured in the frequency range of 100 Hz to 2 kHz. The rock materials were then soaked in water and kerosene (light-oil) respectively, to observe the trends in their attenuation patterns. In the natural rock samples, attenuation increases steadily with frequency for sandstone and glauconite but decreases in shale. In water– saturated rock materials, shale shows no attenuation dispersion which may be due to the pin-points getting easily disrupted by pore-fluid saturation; sandstone shows a decrease from its natural level; which implies a strong dependence of attenuation on pore-fluid; glauconite manifests a step-wise decrease and later increases around 1kHz. The rock samples were saturated with kerosene (light oil) and their characteristic curves changed. Shale completely dissolved making it impossible to observe its attenuation pattern; sandstone attenuates more than glauconite. These results show that pore-fluid in the different rock materials have great influence on the attenuation coefficient of the materials. Key words: Attenuation coefficient, pore-fluid, pulse transmission, sedimentary rocks, seismic waves. 1. Introduction

3


The fact that elastic waves propagating through the earth are attenuated is a common observation. As these elastic waves travel deeper they lose energy unlike in spherical spreading, where energy is spread across a wider area, reflection and transmission of energy occur at boundaries, where redistribution takes place in the upward or downward directions. This energy loss is frequency dependent; meaning that at higher frequencies absorption is more rapid than at lower frequencies. In addition, attenuation appears to vary with the lithology of the medium as suggested by Chopra and Alexeev (2004). Elastic wave attenuation has great potential as a tool to yield a better understanding of the anelastic properties and hence the physical state of rocks in the earth. In consideration of this potential, an expanding body of laboratory work has concentrated on bringing to fruition the diagnostic capabilities of attenuation measurements by Christensen and Wepfer (1989). Two important reasons could be advanced for the need to investigate and understand the attenuation properties of the earth. Firstly, as elastic waves propagate through the subsurface, their amplitudes are reduced. Secondly, attenuation characteristics when determined, could reveal useful information on the type of rock as well as the presence and degree of fluid saturation of rocks. There is an appreciable overlap in the attenuation values of different rocks, but it is evident that sedimentary rocks are generally more absorptive than other types of rock. Amplitude analysis of elastic waves in sedimentary rocks is a common practice in rock Physics. Changes in reservoir seismic properties can be connected with fluid type and changes within the rock as opined by Adams et al. (2009) as well as Gurevich and Lopatnikov (1995). Low-frequency wave propagation in partially saturated rocks is still not well understood because of the lack of precise and reliable experimental techniques. Biot theory (1956) which is the most commonly used theory for studying wave propagation in saturated porous rocks is not without its limitation. This is because the theory estimates attenuation fairly for frequencies higher than seismic frequencies. To resolve the problem of limitation it is very important to develop simple and inexpensive experimental approach to measure attenuation at low frequencies. Our studies analyses wave attenuation in limestone, shale and glauconite of Ewekoro Formation, southwestern Nigeria within the frequency range of 100 Hz to 2 kHz. 2. Theory of Attenuation in Rocks A commonly used measure of attenuation is the attenuation coefficient  , which is the exponential decay constant of the amplitude of a plane wave propagating in a homogeneous medium. The amplitude of this plane wave may be given as

Ar  Ao exp( r )

(1)

Where Ar is the amplitude at any distance r from the source, Ao is the initial or reference amplitude and  is the attenuation coefficient in m-1. Taking natural logarithm of amplitudes will yield ln ( Ar / Ao ) = r = From where



f Qv

f Qv

(2) (3)

4


Where Q is the quality factor of the wave and is a commonly used measure of attenuation, v is the velocity and f is the frequency. The quality factor Q is defined in Dobrin and Savit (1988) as Q

(4)

2 E E For a single cycle therefore, Q

(5)

 (6)  where  is the logarithmic decrement and is defined as the natural logarithm of the ratio of the amplitude of two consecutive cycles, i.e. Q

  ln    

(7)

v

(8)

f

Laboratory measurements of attenuation in rocks show that Q correlates with rock type and fluid type, hence the estimation of Q can be used as a diagnostic tool for rock type discrimination and the effect of fluid. Values of Q for most sedimentary rocks ranges from 20 to 200, according to Sherrif and Geldart (1995). The lower value of Q indicates higher attenuation while higher Q value means the opposite. The spectral ratio technique originally developed by Toksoz et al. (1979) was used to estimate P and S wave attenuation. This procedure assumes that Q is constant over the experimental frequency range and thus it is frequency independent. Hence there is no consideration for dispersion in the spectral ratio model. However, laboratory experiments conducted on several natural rocks have revealed different dispersion and attenuation mechanisms in rocks as caused by intrinsic anelasticity, scattering, and diffraction which can cause changes in velocities and attenuation and as a result affect Q estimates. The accuracy of determining Q across the frequency spectrum is a function of the dispersion characteristics of the waves, which depends on the frequency and the nature of the rocks that disperse the waves. Incorporating the dependence on frequency into attenuation equation (1), leads to Ar  GRAo exp( r ) Ar  GRAo exp( 

fr Qv

(9) )

(10)

where G and R are the geometric spreading factor and the energy partitioning respectively at interface.

5


3.

Experimental Technique Laboratory measurements were carried out on samples of sandstone, glauconite and shale collected from Ewekoro near Abeokuta, Ogun state, Nigeria. These were made available in a prepared form with dimensions 8cm  5cm  3.5cm, for use in the laboratory. The experimental set-up is as shown in Figure 1. The experiment was performed using pulse transmission technique proposed by Johnston and Toksoz (1980) where the amplitude decay of elastic wave signals travelling through a rock sample was measured. Only one way transmission effects were measured. For the fluid-saturation measurement, the samples were immersed in the fluid for 24 hours to ensure the uniformity of saturation. The pulse transmission technique is most suited for use in saturated samples, provided correction can be made for geometric factors such as beam (waves) spreading and reflection. We used the pulse transmission technique and measure attenuation of the sample by using equation (3).

SIGNA L GENERATOR

Y Y C.R.O 2

1

A

A i

i

A

ROCK SAMPLE t

Pair Transducers

of

A t

Figure 1:Experimental setup. The sine-audio signal generator generates the sinusoidal wave, which is passed into the rock sample via transducers in contact with opposite faces of the rock samples. The transducer alloy pair was used to conduct signals from signal generator to the rock sample and from the other face of the rock sample to the Y channel of the oscilloscope. Each plate has a connecting wire soldered to it for wave transmission. Proper care was taken to insulate the rock samples from any unwanted stray signals. The rocks samples were connected electrically by gently pressing with a G-clamp on the transducers to ensure good contact with the rock sample. A double-beam oscilloscope was used to analyze the elastic waves. Its Y1 channel was used to observe the input signal directly 6


from the signal generator while the output from the rock sample was fed into the Y2 channel of the oscilloscope. The amplitudes of the incident (Ai) and transmitted (At) waves were recorded and a measure of attenuation made using the expression in Equation (3). 4.

Results and Discussions Figure 2 shows the plot of attenuation for the dry rock samples. It is observed that attenuation increases steadily with frequency for sandstone. For glauconite, there is a slight drop in attenuation from 100-400 Hz and thereafter increases with frequency. Attenuation in the dry shale sample decreases with frequency to about 1 kHz and shoots up again to 1.5 kHz and decreases further again. Glauconite_dry Shale_dry sandstone_dry

-0.4 -0.6 -0.8

Attenuation

-1.0 -1.2 -1.4 -1.6 -1.8 -2.0 -2.2 -2.4 -2.6 0

500

1000

1500

2000

Frequency Hz

Figure 2: Plot of Attenuation versus Frequency for the natural rock samples. There is clear deviation in the behavior of the rocks from the dry natural condition. This finding is similar to the discovery of Olorode (2001). Shale shows no attenuation dispersion which may be due to the fact that its pin-points get easily disrupted by pore-fluid saturation. Water-saturated sandstone shows an almost opposite response (decrease in attenuation) to that in the natural condition, which means a strong dependence of attenuation dispersion on pore-fluids. This is in line with the findings of Christensen and Wepfer (1989). But for glauconite, the response of the saturated sample shows a stepwise decrease and later increases around 1 kHz.

7


Glauconite_H2O Shale_H20 Sandstone_H20

0.0 -0.2 -0.4 -0.6

Attenuation

-0.8 -1.0 -1.2 -1.4 -1.6 -1.8 -2.0 -2.2 -2.4 -2.6 -2.8 0

500

1000

1500

2000

Frequency

Figure 3: Plot of Attenuation versus Frequency for the water-saturated rock samples. Attenuation in this condition is relatively more than in dry and water-saturated conditions. This is in agreement with previous works by several authors (Wyllie et al., 1962, Gardener et al. 1964, O’Connell and Budiansky, 1978, Winkler and Nur, 1982, Murphy, 1982, Tittmann et al., 1981). It is also observed that attenuation dispersion increases for both rock types across the frequency range of measurement. Hence there is a clear discrimination in the behavior of the two rocks is clearly for water and oil-saturated conditions. Shale completely dissolved in kerosene and as a result it was not possible to study its attenuation dispersion when saturated with this light oil.

8


Glauconite_oil Sandstone_oil

-1.3 -1.4 -1.5

Attenuation

-1.6 -1.7 -1.8 -1.9 -2.0 -2.1 -2.2 -2.3 -2.4 0

500

1000

1500

2000

Frequency Hz

Figure 4 :

Attenuation versus Frequency of kerosene-saturated sandstone and glauconite

5.

Conclusion The attenuation characteristics of sandstone, shale and glauconite of Ewekoro were presented. The three rock types behaved differently to different pore-fluid saturation. At high frequencies, attenuation is higher than at lower frequencies. In the natural rock samples, sandstone attenuates most followed by shale and the least was glauconite. This is in agreement with the results obtained by Olorode (2001) and Umo (1998). When the rock samples were water-saturated, shale attenuates most followed by glauconite and sandstone was the least attenuated. However, when in kerosene (light oil) , sandstone attenuates most, followed by glauconite while shale dissolved completely. Pore-fluid in the rock matrices has a great influence on the attenuation pattern of the rock materials.

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References Adam, L., M.Batzle, K. T. Lewallen, and van Wijk, K. (2009). “Seismic wave attenuation in carbonates.”Journal of Geophysical Research. 114, pp. B06208. doi: 10.1029/2008JB005890. Biot, M. A. (1962) “Generalized theory of Acoustic propagation in porous dissipative media” J. Acoust. Soc. Am. 34 pp 1254 - 1264 Chopra, S. and V. Alexev (2004). “A new approach to enhancement of frequency bandwidth of surface seismic data.” First break. 22, pp. 31-42. Christensen, N.I. and W. W. Wepfer (1989) “Laboratory techniques for determining seismic velocities and attenuations, with applications to the continental lithosphere,” in Pakiser, L.C., and Mooney, W.D., Geophysical framework of the continental United States: Boulder, Colorado, Geophysical Society of America Memoir 172, pp. 91-102. Frempong, P., Butt, S., and Donald, A. (2005). “Frequency dependent spectral ratio technique for Q estimate.” Rainbow in the Earth, 2nd International Workshop, Lawrence Berkeley National laboratory, Berkeley, California. Gardner, G.H.F., Wyllie, M.P.J., and Droschak, D.M. (1964). Effects of pressure and fluid-saturation on the attenuation of elastic waves in sand. Journal of Petroleum Technology, vol. 16, pp. 189-198. Gurevich, B. and S. L. Lopatnikov (1995) “ Velocity and Attenuation of Elastic waves in finely layered Porous rocks” Geoph. J. Int. 121 pp 933 - 947 Murphy, W.F., III. (1982). Effects of partial water saturation on attenuation in Massilon sandstone and Vycor porous glass. Journal of the Acoustical Society of America, vol. 71, pp. 1458-1468. O’Connell, R.J. and B. Budiansky (1978). “Measure of dissipation in viscoelastic media”. Geophysical Research letters, vol. 5, pp. 5-8. Olorode, D. O. (2001) “ Analysis of the Temperature effects on the Attenuation Coefficients and anelastic properties of three Sedimentary rocks from South Western Nigeria” Ph. D.Thesis University of Ibadan. Nigeria. Shankland, T.J., P. A. Johnson and T. M. Hopson (1993) “Elastic wave attenuation and velocity of Berea sandstone measured in the frequency domain”. Geophysical research Letters. 20 (5), pp. 391-394. Sheriff, R.G. and L. P. Geldart (1995) “Exploration Seismology (2nd ed.)”. Cambridge University Press.

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Tittman, B.R., H. Nadler, V. A. Clark, L. A. Ahlberg and T. W. Spencer (1981) “Frequency dependence of seismic dissipation in saturated rocks,” Geophysical Research Letters, 38, pp. 89-94. Toksoz, M.N., D. H. Johnston and A. Timur (1979). “Attenuation of seismic waves in dry and saturated rocks; 1, Laboratory measurements”. Geophysics, 44, pp. 681-690. Umo, J. A. (1998) “ Analysis of the wave Amplitudes of broadband seismic waveforms propagated through different geological samples from Abeokuta and Ewekoro formations, Eastern Dahomey Basin” Ph.D. Thesis, University of Ibadan. Nigeria. Winkler, K.W. and A. Nur (1979). “Pore fluids and seismic attenuation in rocks.” Geophysical Research Letters 87, pp. 1-15. Wyllie, M.R.J., G. H. F. Gardner and A.R. Gregory (1962). “Studies of elastic wave attenuation in porous media” Geophysics, 27, pp. 569-589.

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Good’s syndrome (thymoma with immunodeficiency): An unusual clinical presentation of bulbar symptoms and peripheral neuropathy Nevein PB Clinical School Johor Bahru, Jeffrey Cheah School of Medicine and Health Sciences, Monash University, Sunway campus, JKR 1235 Bukit Azah, 80100 Johor Bahru, Johor, Malaysia, [email protected]

Kan FK Department of Medicine, Hospital Sultanah Aminah, 80100 Johor Bahru, Johor, Malaysia, [email protected]

Amgad AR Clinical School Johor Bahru, Jeffrey Cheah School of Medicine and Health Sciences, Monash University, Sunway campus, JKR 1235 Bukit Azah, 80100 Johor Bahru, Johor, Malaysia, [email protected]

Shanthi V Department of Neurology, Hospital Kuala Lumpur, 50586 Jalan Pahang, Wilayah Persekutuan, Kuala Lumpur, Malaysia, [email protected]

Rusli BN Clinical School Johor Bahru, Jeffrey Cheah School of Medicine and Health Sciences, Monash University, Sunway campus, JKR 1235 Bukit Azah, 80100 Johor Bahru, Johor, Malaysia, [email protected]

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Abstract We report a rare case of Good’s syndrome (thymoma with immunodeficiency) with unusual neurological manifestations. A 52 year-old lady, with previous history of pulmonary tuberculosis, presented to the hospital with recurrent respiratory infections. Subsequently, she developed bulbar symptoms, limb weakness, and numbness. Nerve conduction velocity study showed mixed sensorimotor axonal polyneuropathy. Computerized tomography (CT) scan and magnetic resonance imaging (MRI) of the brain did not show significant lesions. The possible etiological explanations would be one of possible paraneoplastic association as the patient has a thymoma, autoimmune or post infectious cause. The patient was treated with regular IV immunoglobulin with no improvement in her polyneuropathic condition. Keywords: Good’s syndrome, thymoma, hypogammaglobulinemia, immunodeficiency, polyneuropathy Background Dr Robert A Good first reported the association between hypogammaglobulinemia and increased susceptibility to infections in 1954.1 He also described the association between thymoma and hypogammaglobulinemia in 1956.2 Good’s syndrome (thymoma with immunodeficiency) is a rare disease of combined B and T cell immunodeficiency in adults with increased susceptibility to infection by bacterial, opportunistic viral and fungal infections.3 The most consistent immunological abnormalities are hypogammaglobulinemia and reduced or absent B cells. Thymomas are associated with a variety of paraneoplastic syndromes. Although some of these manifestations, such as pure red cell aplasia, improved by thymectomy, others such as hypogammaglobulinemia, may not be influenced by surgical resection.4 Case Presentation A 52 year-old lady presented to the hospital with chronic productive cough, fever, weakness and weight loss. Her illness began in 1992 when she was diagnosed with pulmonary tuberculosis and completed treatment. Since then, she has had recurrent airway infections. A CT scan of the thorax in 2002 to exclude bronchiectasis revealed a thymoma. Preoperative assessment revealed a severe symptomatic anaemia (haemoglobin of 2.8 gm/dl). She was found to have red cell hypoplasia by bone marrow examination. Preoperatively, she was given immunoglobulin and antibiotics. In August 2002, thymectomy was done and the histopathological examination of the resected specimen revealed a “well-capsulated tumour, composed of sheets of cells with uniform ovoid to spindle vesicular nuclei mixed with mature lymphocytes. In places, the cells show rosette-like pattern. The cells are positive for cytokeratin. Impression: anterior mediastinal tumour (thymoma).” Post-operative recovery was uneventful and red cell hypoplasia resolved. In 2004, she started to have multiple hospital admissions with recurrent fever, productive cough with mucopurulent sputum and breathlessness, each episode’s sputum culturing different microorganisms (Pseudomonas aeroginosa, Klebsiella pneumonia, and Hemophilus parainfluenza). Tuberculosis work-up was negative in both 2005 and 2006. High contrast CT in December 2006 showed pulmonary fibrosis with mild bronchiolar dilatation.

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In December 2009, she presented with rash for one month. She was emaciated with generalized dry skin and photo distributed maculopapular eruption and erythematous plaque on the nose. She had multiple excoriations and excoriated papules as well as a vesicle. Skin biopsy showed a skin vesicle with multinucleated giant cell and balloon degeneration suggestive of herpes simplex virus infection and the lesions were recurrent. There was no evidence to suggest polymyositis or sarcoidosis. Creatine kinase and serum calcium levels were normal. In early 2010, she developed progressive hoarseness of voice, dysphagia to solid food, nasal fluid regurgitation and occasional choking. Examination of the ear, nose and throat showed atrophic changes of the tongue, pooling of saliva, bilateral vocal cord palsy and absent gag reflex. Oesophagogastroscopy showed no abnormalities. She was advised for Ryle’s tube feeding but declined till now. Later in 2010, she was given a short course of prednisolone for suspected sarcoidosis. Subsequently, she developed blurring of vision of the right eye. An ophthalmologist suspected cytomegalovirus (CMV) retinitis but we were unable to send vitreous for CMV viral load due to logistic limitations. She was given intravitreal gancyclovir repeatedly till now. She also received intravenous gancyclovir twice (each time 2 weeks). Initially, retinal changes improved slightly but subsequently deteriorated quickly. Now she only has light perception bilaterally. Human immunodeficiency virus (HIV) screening and anti-nuclear antibody (ANA) levels were negative. C3 level was 1.26 g/L (normal range: 0.9-1.8 g/L) whilst the C4 level was 0.48 g/L (normal range: 0.1-0.4 g/L). Rheumatoid factor was negative and thyroid function tests were normal. The following were sero-immunological findings reported between August 2010 and December 2011 (Appendix 1: Table 1). In 2011, she started to have progressive weakness and numbness in all four limbs. Neurological examination showed muscle wasting with widespread muscle weakness, which was predominantly distal; all limb reflexes were absent and the planters were down going. Nerve conduction velocity study was arranged and showed evidence of sensorimotor polyneuropathy of axonal nature (Appendix 2A: Table 2A; Appendix 2B: Table 2B). Needle electromyogram showed diffuse neurogenic process in all muscles tested including the first dorsal interrossei, deltoids, tibialis anterior, medial gastrocnemius, quadriceps femoris bilaterally and genioglossus characterized by high firing, spontaneous activity, occasional fasciculation and neurogenic motor unit potentials with broad motor unit potentials, increased duration, polyphasia and reduced recruitment (Appendix 3: Figure 1). Sural nerve biopsy and lumbar puncture was not done as patient refused invasive intervention. On follow-up (for 16 months), there was no improvement in the patient’s condition. Acetylcholine receptor antibodies and Anti-Hu, Anti-Ro and Anti-Ri antibodies were negative. CT of the chest excluded recurrence of the thymoma. Whole body scan excluded the presence of any malignancy. Discussion A review of the literature and systematic summary of information from 152 patients with Good’s syndrome by Kelesidis and Yang showed that the mean age of the

14


patients was 59.1 years at the time of the first manifestation (thymoma, infection or hypogammaglobulinemia). The diagnosis of thymoma preceded the diagnosis of hypogammaglobulinemia, infection, or diarrhoea in 42.4% of the patients. Thymoma was diagnosed after the documentation of infection or hypogammaglobulinemia in 19.7% of the cases with an interval of 3 months to 15 years. In 37.9% of patients, the diagnoses were made almost simultaneously within 2 months of each other and in 8 patients, the thymoma was only diagnosed at autopsy.6 Laboratory findings Kelesidis and Yang reported the following6: Anemia (85%) (patients may have normal haemoglobin) Leukopenia (46.5%) or leucocytosis (18.6%) (normal white cell count in some patients) Neutropenia (15.1%) Thrombocytopenia (20%) or thrombocytosis Absence of eosinophil (19.8%) Lymphocytopenia (35.1%) (normal lymphocyte count can be found) Low B cells (83%) or absent peripheral B cells (87%) Low T cells (87%) Low CD4+ count (15%) A high CD8+ count (55%), normal CD8+ count (35%) and low CD8+ count (10%) Low CD4+/CD8+ ratio (76.1%) Low NK cells (57.1%) Hypogammaglobulinemia (100%) Infections Kelesidis and Yang6 found that the most common infection is recurrent infection of the respiratory tract (upper and lower). The most common pathogens were Hemophilus influenza and Pseudomonas spp. CMV and Candida spp were the most common opportunistic pathogens reported.6 Diarrhoea Diarrhoea (especially chronic) is present in almost 50% of patients with Good’s syndrome. Paraneoplastic neurological syndromes and autoimmune manifestations Thymomas are associated with different paraneoplastic syndromes: myasthenia gravis, Lambert-Eaton myasthenic syndrome, pemphigus, subacute sensory neuropathy, pure red cell aplasia, and immunodeficiency. Most of these are autoimmune or endocrinerelated. The common manifestations are myasthenia gravis, pure red cell aplasia, and hypogammaglobulinemia. Here, we report a new finding with Good’s syndrome which is the presence of axonal sensorimotor polyneuropathy, evidenced clinically by limb numbness and weakness, and generalized wasting of the limb muscles. The clinical findings were

15


confirmed by nerve conduction velocity studies, and the electromyogram showed diffuse neurogenic changes. Repetitive nerve stimulation was normal, thereby excluding the presence of myasthenia gravis and Lambert-Eaton syndrome. Management of Good's syndrome Thymectomy The treatment of thymoma is surgical removal or debulking of the tumour. Thymectomy can prevent locally invasive growth and metastasis of thymomas and usually has a favourable effect on associated conditions such as myasthenia gravis and pure red cell aplasia but does not usually reverse the immunological abnormalities.6 We had a recent CT thorax done showing no residual thymoma. We couldn’t do MRI as she was unable to lie flat for prolonged periods due to respiratory distress. Gamma globulin Immunoglobulin replacement treatment has been reported to improve infection control, reduce hospitalization, and decrease use of antibiotics. It was noticed that the incidence of infections after treatment with gamma globulin is reduced.6 Conclusions In the case reported here, thymoma was discovered after repeated chest infections, presence of immunodeficiency and recurrent infections remaining after thymectomy while the red cell hypoplasia recovered completely. The neurological findings suggest a subacute onset of diffuse axonal type of sensorimotor polyneuropathy with bulbar involvement as a neurological manifestation under the spectrum of Good’s syndrome. The possible etiological explanations would be one of possible paraneoplastic association as the patient has a thymoma, autoimmune or post infectious cause. The patient was treated with regular IV immunoglobulin with no improvement in her polyneuropathic condition. It is highly likely this is a de-novo manifestation of this disease which needs to be recognized and looked out for in patients. However, etiological explanation for it is difficult at present. Acknowledgements We acknowledge, with gratitude, the permission granted by the Director, Hospital Sultanah Aminah to publish the study. We are indebted to the patient and her daughter for providing informed consent for the study and publication.

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References 1

Good RA. Agammaglobulinaemia - a provocative experiment of nature. Bulletin of the University of Minnesota 1954; 26:1–19. http://www.robertagoodarchives.com/images/00330001.pdf. 2

Maclean LD, Varco RL, Good RA, et al. Thymic tumor and acquired agammaglobulinemia: a clinical and experimental study of the immune response. Surgery 1956; 40:1010. http://www.robertagoodarchives.com/images/00590001.pdf. 3

Kelleher P. What is Good’s syndrome? Immunological abnormalities in patients with thymoma. Clinical Pathology 2003; 56:12–16. 4

van der Marel J, Pahlplatz PVM, Steup W-H, Hendriks ER. Thymoma with paraneoplastic syndromes, Good’s syndrome, and pure red cell aplasia. Journal of Thoracic Oncology 2007; 2:325–326. 5

Samson M. Diagnostic strategy for patients with hypogammaglobulinemia in rheumatology. Joint Bone Spine 2011; 78:241–245. 6

Kelesidis T, Yang O. Good's syndrome remains a mystery after 55 years: a systematic review of the scientific evidence. Clinical Immunology 2010; 135:347363. 7

Primary Immunodeficiency Diseases. Report of an IUIS Scientific Committee. Clinical and Experimental Immunology 1999; 118 (Suppl 1):1–28.

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Appendix 1 Table 1: Sero-immunological findings December 2010

December 2011

CD3 (normal range: 1117-2250 cells/µL)

1141

867


290

232


753

589

0.385%

0.393%

CD4/CD8

September 2010 IgA: (normal range: 0.68-3.76 g/l)

0.77

IgM: (normal range: 0.60-2.63 g/l)

< 0.20

IgG: (normal range: 7.23-16.85 g/l)

5.13

Total IgE: (normal range: