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extrapontine myelinolysis: A case report
Progress in Neuro-Psychopharmacology & Biological Psychiatry 32 (2008) 1344-1346
Contents lists available at ScienceDirect
Progress in Neuro-Psychopharmacology & Biological Psychiatry j o u r n a l h o m e p a g e : w w w. e l s e v i e r. c o m / l o c a t e / p n p b p
Letter to the Editor (Case report)
Catatonic syndrome in central pontine/extrapontine myelinolysis: A case report
1. Introduction Adams et al. (1959) first described central pontine myelinolysis (CPM) in those with alcohol dependence and malnutrition. With 10%–60% cases showing extrapontine myelinolysis (EPM) involving cerebellum, caudate and lentiform nuclei, internal and external capsule, lateral geniculate body, hippocampus, putamen, cerebral cortex, or thalamus, the syndrome was renamed as Central pontine and extrapontine myelinolysis (CPEM) (Uchino et al., 2003; Martin and Brown, 2004). The non-specific neurological manifestations of CPM include weakness of face and tongue, difficulty in swallowing and speaking, quadriplegia, pseudobulbar palsy and encephalopathy or coma (Illowsky and Laureno, 1993; Brown, 2000). The EPM is characterized by tremor and ataxia, parkinsonism, dystonia, choreoathetosis, cogwheel rigidity, dysarthria, dystonia, gait disturbance, mutism, myoclonus and movement disorders (Brown, 2000). The neurobehavioral manifestations include personality change, inappropriate affect, emotional lability or incontinence, disinhibition, poor judgment, paranoia, delirium, and schizophrenia like psychosis (Illowsky and Laureno, 1993; Price and Mesulam, 1987). Transient pyramidal signs followed by confusion, restless behavior, pressured tangential speech, and disinhibition have been reported. The catatonic syndrome, reported as a rare manifestation may be due to EPM (Chalela and Kattah, 1999; Koussa and Nasnas, 2003). We describe a middle-aged man with affective disorder who, following rapid correction of hyponatremia, developed catatonia and other neurological symptoms diagnosed as CPEM and followed by a complete neurological recovery.
hospital. On admission his heamogram, serum electrolytes, and liver and renal function tests were all normal except for hyponatremia (serum sodium 107 mmol/L), which was corrected with 3% normal saline. Over the next 36 h he stopped vomiting but developed difficulty in swallowing food, incontinence of urine, nasal intonation of speech and slowness of movements. His first psychiatric consultation then led to prescription of Tablet Escitalopram 10 mg/day. At the end of 5 days of hospitalization his speech and swallowing improved slightly, but other symptoms continued. Four days after starting Tablet Escitalopram, he suddenly developed hypomanic symptoms without any cognitive deficits or diurnal variation. Within a day Escitalopram was stopped. Over the next 4 days his hypomania abated but neurological symptoms worsened. He developed generalized stiffness and slowing of body, reduced eye blinking, drooling of saliva, nasal regurgitation of fluids, posturing, perseveration, refusal to eat, and speech output reduced to answering only in yes/no, and he needed support for all daily activities. There was no flexor spasm, difficulty in walking or getting up from squatting position.
2. The case A 52-year pre-morbidly well-adjusted male with no substance abuse or past or family history of medical or psychiatric illness was admitted for sudden onset inability to eat, talk and walk along with generalized stiffness of body and slowness. His present illness started 8 months back, when he developed an episode of major depression (as per DSM-IV). In first 6 months, off and on vitamin supplements led to no improvement. Later, following an episode of heaviness of chest he was diagnosed with hypertension and prescribed unspecified medication. On this medication, the next day, he developed watery, copious, non-bilious, and projectile vomiting which was not associated with food intake, hematemesis or pain in the abdomen. Over the next 4 weeks despite taking antiemetics the frequency of vomiting increased from 2–3 to 8–10 times daily and he became slow in activities like walking and talking, and gradually progressed to a bedridden state necessitating emergency admission in another 0278-5846/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.pnpbp.2008.03.009
Fig. 1. MRI showing hyperintense T2 weighted image involving central pons and bilateral putamen suggestive of osmotic myelinosis.
S.K. Mattoo et al. / Progress in Neuro-Psychopharmacology & Biological Psychiatry 32 (2008) 1344-1346
1345
Fig. 2. Follow up MRI after 20 months of the hyponatremia showing residual signal changes, involving central pons only.
Brought to emergency services of our hospital, and he was later admitted to psychiatry ward with a diagnosis of catatonia (Bush Francis Catatonia Scale score 12). His physical examination revealed masked facies, staring gaze, mutism and increased jaw jerk. In all limbs power was 3/5, along with hypertonia, generalized rigidity, increased (3–4+) deep tendon reflexes, ankle clonus and flexor planters. The rigidity in lower limbs was lead pipe and in upper limbs cogwheel type. He also had waxy flexibility and automatic obedience. His heamogram, liver and renal function tests, serum electrolytes (including sodium, potassium, calcium, chloride and magnesium), blood glucose levels, cerebrospinal fluid examination, routine urine examination, electroencephalogram, electro-cardiogram were within normal limits. His blood culture and examination for malarial parasite also did not reveal any infective pathology. His magnetic resonance imaging (MRI) revealed altered signals, which appeared hypointense on T1 weighted imaging and hyperintense on T2 weighted imaging and on flair involving central pons and bilateral putamen suggestive of osmotic myelinosis (Fig. 1). His supportive management included Ryle's tube feeding. Intravenous Lorazepam up to 6 mg daily did not improve neurological symptoms; instead he developed tremors and multiple myoclonic jerks. Two weeks later he was transferred from psychiatry to neurology ward. Over the next week, management with abdominal gastrostomy feeding, Levodopa 150 mg, Carbidopa 37.5 mg/day in divided doses and baclofen 10 mg/day, led to partial improvement in neurological symptoms. His family was explained the nature of the illness, and the need for occupational, physical, and speech-language therapies in the future. He was discharged with final diagnoses of CPEM and Organic bipolar affective disorder. Over the next month, Syndopa and Baclofen were tapered off as his neurological symptoms improved remarkably. He was able to move around without help. As he started eating enough his gastrostomy was removed. His psychometric evaluation did not reveal any evidence of cognitive deficits. During the follow up over the next 6 months, he manifested affective symptoms amounting to major depression, followed by hypomania (as per DSM-IV). He was started on Tab Sodium valproate up to 1500 mg/day. His repeat MRI revealed residual signal changes (Fig. 2), involving central pons with no other abnormality.
3. Discussion In our case the catatonic syndrome was attributed to CPEM induced by serum osmotic changes and not the affective disorder because of the evidence of hyponatremia, neurological symptoms developing immediately after correction of hyponatremia, and the presence of neurological symptoms during both depressive and hypomanic phases. Further, the onset of typical catatonic signs like generalized stiffness and slowing of body, reduced eye blinking, posturing, and perseveration occurred when he was coming out of hypomania and there were no depressive symptoms during that time. Overall, catatonia is no more considered only as a subtype of schizophrenia and many other etiologies have been suggested (Cottencin et al., 2007). However, catatonia seems to be a relatively rare manifestation in CPEM as reflected by only 2 case report publications (Chalela and Kattah, 1999; Koussa and Nasnas, 2003). The excellent radiological and clinical recovery in our case is similar to that reported in a few cases (Chemaly et al., 1998; Nagamitsu et al., 1999), including the one not caused by hyponatremia (Bernsen and Prick, 1999). Thus, our case highlights the need for detailed evaluation for organic causes in all patients presenting with catatonic syndrome and other neurological manifestations, especially in the context of possible rapid serum osmotic changes. References Adams RD, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholic and malnourished patients. AMA Arch Neurol Psychiatry 1959;81:154–72. Bernsen HJ, Prick MJ. Improvement of central pontine myelinolysis as demonstrated by repeated magnetic resonance imaging in a patient without evidence of hyponatremia. Acta Neurol Belg 1999;99:189–93. Brown WD. Osmotic demyelination disorders: central pontine and extrapontine myelinosis. Curr Opin Neurol 2000;13:691–7. Chalela J, Kattah J. Catatonia due to central pontine and extrapontine myelinolysis: case report. J Neurol Neurosurg Psychiatry 1999;67:692–3. Chemaly R, Halaby G, Mohasseb G, et al. Myelinolyse extra-pontine: traitment par TRH. Rev Neurol 1998;154:163–5. Cottencin O, Warembourg F, de Chouly de Lenclave MB, Lucas B, Vaiva G, Goudemand M, et al. Catatonia and consultation-liaison psychiatry study of 12 cases. Prog Neuropsychopharmacol Biol Psychiatry 2007;31:1170–6.
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S.K. Mattoo et al. / Progress in Neuro-Psychopharmacology & Biological Psychiatry 32 (2008) 1344-1346
Illowsky B, Laureno R. Pontine and extrapontine myelinolysis. Pontine and extrapontine myelinolysis: a neurologic disorder following rapid correction of hyponatremia. Medicine 1993;72:359–73. Koussa S, Nasnas R. Catatonia and Parkinsonism due to extrapontine myelinolysis following rapid correction of hyponatremia: a case report. J Neurol 2003;250: 103–5. Martin RJ, Brown WD. Central pontine and extrapontine myelinosis: the osmotic demyelination syndromes. J Neurol Neurosurg Psychiatry 2004;75(Suppl III): iii22–8. Nagamitsu S, Matsuishi T, Yamashita Y, Yamada S, Kato H. Extrapontine myelinolysis with Parkinsonism after rapid correction of hyponatremia: high cerebrospinal fluid level of homovanillic acid and successful dopaminergic treatment. J Neural Transm 1999;106:949–53. Price BH, Mesulam MM. Behavioral manifestations of central pontine myelinolysis. Arch Neurol 1987;44:671–3. Uchino A, Yuzuriha T, Murakami M, Endoh K, Hiejima S, Koga H, et al. Magnetic resonance imaging of sequelae of central pontine myelinosis in chronic alcohol abusers. Neuroradiology 2003;45:877–80.
Surendra Kumar Mattoo* Parthasarathy Biswas Madhusmita Sahoo Sandeep Grover Department of Psychiatry, Post Graduate Institute of Medical Education & Research, Chandigarh, 160012, India *Corresponding author. Tel.: +91 172 2756813; fax: +91 172 2744401. E-mail address: [email protected] (S.K. Matto).
5 February 2008 Available online xxx
Contents lists available at ScienceDirect
Progress in Neuro-Psychopharmacology & Biological Psychiatry j o u r n a l h o m e p a g e : w w w. e l s e v i e r. c o m / l o c a t e / p n p b p
Letter to the Editor (Case report)
Catatonic syndrome in central pontine/extrapontine myelinolysis: A case report
1. Introduction Adams et al. (1959) first described central pontine myelinolysis (CPM) in those with alcohol dependence and malnutrition. With 10%–60% cases showing extrapontine myelinolysis (EPM) involving cerebellum, caudate and lentiform nuclei, internal and external capsule, lateral geniculate body, hippocampus, putamen, cerebral cortex, or thalamus, the syndrome was renamed as Central pontine and extrapontine myelinolysis (CPEM) (Uchino et al., 2003; Martin and Brown, 2004). The non-specific neurological manifestations of CPM include weakness of face and tongue, difficulty in swallowing and speaking, quadriplegia, pseudobulbar palsy and encephalopathy or coma (Illowsky and Laureno, 1993; Brown, 2000). The EPM is characterized by tremor and ataxia, parkinsonism, dystonia, choreoathetosis, cogwheel rigidity, dysarthria, dystonia, gait disturbance, mutism, myoclonus and movement disorders (Brown, 2000). The neurobehavioral manifestations include personality change, inappropriate affect, emotional lability or incontinence, disinhibition, poor judgment, paranoia, delirium, and schizophrenia like psychosis (Illowsky and Laureno, 1993; Price and Mesulam, 1987). Transient pyramidal signs followed by confusion, restless behavior, pressured tangential speech, and disinhibition have been reported. The catatonic syndrome, reported as a rare manifestation may be due to EPM (Chalela and Kattah, 1999; Koussa and Nasnas, 2003). We describe a middle-aged man with affective disorder who, following rapid correction of hyponatremia, developed catatonia and other neurological symptoms diagnosed as CPEM and followed by a complete neurological recovery.
hospital. On admission his heamogram, serum electrolytes, and liver and renal function tests were all normal except for hyponatremia (serum sodium 107 mmol/L), which was corrected with 3% normal saline. Over the next 36 h he stopped vomiting but developed difficulty in swallowing food, incontinence of urine, nasal intonation of speech and slowness of movements. His first psychiatric consultation then led to prescription of Tablet Escitalopram 10 mg/day. At the end of 5 days of hospitalization his speech and swallowing improved slightly, but other symptoms continued. Four days after starting Tablet Escitalopram, he suddenly developed hypomanic symptoms without any cognitive deficits or diurnal variation. Within a day Escitalopram was stopped. Over the next 4 days his hypomania abated but neurological symptoms worsened. He developed generalized stiffness and slowing of body, reduced eye blinking, drooling of saliva, nasal regurgitation of fluids, posturing, perseveration, refusal to eat, and speech output reduced to answering only in yes/no, and he needed support for all daily activities. There was no flexor spasm, difficulty in walking or getting up from squatting position.
2. The case A 52-year pre-morbidly well-adjusted male with no substance abuse or past or family history of medical or psychiatric illness was admitted for sudden onset inability to eat, talk and walk along with generalized stiffness of body and slowness. His present illness started 8 months back, when he developed an episode of major depression (as per DSM-IV). In first 6 months, off and on vitamin supplements led to no improvement. Later, following an episode of heaviness of chest he was diagnosed with hypertension and prescribed unspecified medication. On this medication, the next day, he developed watery, copious, non-bilious, and projectile vomiting which was not associated with food intake, hematemesis or pain in the abdomen. Over the next 4 weeks despite taking antiemetics the frequency of vomiting increased from 2–3 to 8–10 times daily and he became slow in activities like walking and talking, and gradually progressed to a bedridden state necessitating emergency admission in another 0278-5846/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.pnpbp.2008.03.009
Fig. 1. MRI showing hyperintense T2 weighted image involving central pons and bilateral putamen suggestive of osmotic myelinosis.
S.K. Mattoo et al. / Progress in Neuro-Psychopharmacology & Biological Psychiatry 32 (2008) 1344-1346
1345
Fig. 2. Follow up MRI after 20 months of the hyponatremia showing residual signal changes, involving central pons only.
Brought to emergency services of our hospital, and he was later admitted to psychiatry ward with a diagnosis of catatonia (Bush Francis Catatonia Scale score 12). His physical examination revealed masked facies, staring gaze, mutism and increased jaw jerk. In all limbs power was 3/5, along with hypertonia, generalized rigidity, increased (3–4+) deep tendon reflexes, ankle clonus and flexor planters. The rigidity in lower limbs was lead pipe and in upper limbs cogwheel type. He also had waxy flexibility and automatic obedience. His heamogram, liver and renal function tests, serum electrolytes (including sodium, potassium, calcium, chloride and magnesium), blood glucose levels, cerebrospinal fluid examination, routine urine examination, electroencephalogram, electro-cardiogram were within normal limits. His blood culture and examination for malarial parasite also did not reveal any infective pathology. His magnetic resonance imaging (MRI) revealed altered signals, which appeared hypointense on T1 weighted imaging and hyperintense on T2 weighted imaging and on flair involving central pons and bilateral putamen suggestive of osmotic myelinosis (Fig. 1). His supportive management included Ryle's tube feeding. Intravenous Lorazepam up to 6 mg daily did not improve neurological symptoms; instead he developed tremors and multiple myoclonic jerks. Two weeks later he was transferred from psychiatry to neurology ward. Over the next week, management with abdominal gastrostomy feeding, Levodopa 150 mg, Carbidopa 37.5 mg/day in divided doses and baclofen 10 mg/day, led to partial improvement in neurological symptoms. His family was explained the nature of the illness, and the need for occupational, physical, and speech-language therapies in the future. He was discharged with final diagnoses of CPEM and Organic bipolar affective disorder. Over the next month, Syndopa and Baclofen were tapered off as his neurological symptoms improved remarkably. He was able to move around without help. As he started eating enough his gastrostomy was removed. His psychometric evaluation did not reveal any evidence of cognitive deficits. During the follow up over the next 6 months, he manifested affective symptoms amounting to major depression, followed by hypomania (as per DSM-IV). He was started on Tab Sodium valproate up to 1500 mg/day. His repeat MRI revealed residual signal changes (Fig. 2), involving central pons with no other abnormality.
3. Discussion In our case the catatonic syndrome was attributed to CPEM induced by serum osmotic changes and not the affective disorder because of the evidence of hyponatremia, neurological symptoms developing immediately after correction of hyponatremia, and the presence of neurological symptoms during both depressive and hypomanic phases. Further, the onset of typical catatonic signs like generalized stiffness and slowing of body, reduced eye blinking, posturing, and perseveration occurred when he was coming out of hypomania and there were no depressive symptoms during that time. Overall, catatonia is no more considered only as a subtype of schizophrenia and many other etiologies have been suggested (Cottencin et al., 2007). However, catatonia seems to be a relatively rare manifestation in CPEM as reflected by only 2 case report publications (Chalela and Kattah, 1999; Koussa and Nasnas, 2003). The excellent radiological and clinical recovery in our case is similar to that reported in a few cases (Chemaly et al., 1998; Nagamitsu et al., 1999), including the one not caused by hyponatremia (Bernsen and Prick, 1999). Thus, our case highlights the need for detailed evaluation for organic causes in all patients presenting with catatonic syndrome and other neurological manifestations, especially in the context of possible rapid serum osmotic changes. References Adams RD, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholic and malnourished patients. AMA Arch Neurol Psychiatry 1959;81:154–72. Bernsen HJ, Prick MJ. Improvement of central pontine myelinolysis as demonstrated by repeated magnetic resonance imaging in a patient without evidence of hyponatremia. Acta Neurol Belg 1999;99:189–93. Brown WD. Osmotic demyelination disorders: central pontine and extrapontine myelinosis. Curr Opin Neurol 2000;13:691–7. Chalela J, Kattah J. Catatonia due to central pontine and extrapontine myelinolysis: case report. J Neurol Neurosurg Psychiatry 1999;67:692–3. Chemaly R, Halaby G, Mohasseb G, et al. Myelinolyse extra-pontine: traitment par TRH. Rev Neurol 1998;154:163–5. Cottencin O, Warembourg F, de Chouly de Lenclave MB, Lucas B, Vaiva G, Goudemand M, et al. Catatonia and consultation-liaison psychiatry study of 12 cases. Prog Neuropsychopharmacol Biol Psychiatry 2007;31:1170–6.
1346
S.K. Mattoo et al. / Progress in Neuro-Psychopharmacology & Biological Psychiatry 32 (2008) 1344-1346
Illowsky B, Laureno R. Pontine and extrapontine myelinolysis. Pontine and extrapontine myelinolysis: a neurologic disorder following rapid correction of hyponatremia. Medicine 1993;72:359–73. Koussa S, Nasnas R. Catatonia and Parkinsonism due to extrapontine myelinolysis following rapid correction of hyponatremia: a case report. J Neurol 2003;250: 103–5. Martin RJ, Brown WD. Central pontine and extrapontine myelinosis: the osmotic demyelination syndromes. J Neurol Neurosurg Psychiatry 2004;75(Suppl III): iii22–8. Nagamitsu S, Matsuishi T, Yamashita Y, Yamada S, Kato H. Extrapontine myelinolysis with Parkinsonism after rapid correction of hyponatremia: high cerebrospinal fluid level of homovanillic acid and successful dopaminergic treatment. J Neural Transm 1999;106:949–53. Price BH, Mesulam MM. Behavioral manifestations of central pontine myelinolysis. Arch Neurol 1987;44:671–3. Uchino A, Yuzuriha T, Murakami M, Endoh K, Hiejima S, Koga H, et al. Magnetic resonance imaging of sequelae of central pontine myelinosis in chronic alcohol abusers. Neuroradiology 2003;45:877–80.
Surendra Kumar Mattoo* Parthasarathy Biswas Madhusmita Sahoo Sandeep Grover Department of Psychiatry, Post Graduate Institute of Medical Education & Research, Chandigarh, 160012, India *Corresponding author. Tel.: +91 172 2756813; fax: +91 172 2744401. E-mail address: [email protected] (S.K. Matto).
5 February 2008 Available online xxx