|Year : 2015 | Volume
| Issue : 1 | Page : 103-105
Hyperhomocysteinemia presenting as Wallenberg syndrome: A report of two cases and review of literature
Puneet Chabbra1, Nikhil Gupta2, Arun Garg2, Sakshi Khurana3
1 2nd year Resident, Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Medicine, University College of Medical Sciences, New Delhi, India
3 Department of Radiodiagnosis, University College of Medical Sciences, New Delhi, India
|Date of Web Publication||8-Jan-2015|
Department of Medicine, University College of Medical Sciences, New Delhi - 110 095
Source of Support: None, Conflict of Interest: None
Wallenberg syndrome or lateral medullary syndrome occurs due to infarction of the lateral medulla usually supplied by the posterior inferior cerebellar artery (PICA). It most commonly results from occlusion of the vertebral artery followed by the PICA. The clinical manifestations result from impaired functioning of vestibular nuclei, inferior cerebellar peduncle, central tegmental tract, descending sympathetic fibers, nucleus ambiguus, spinothalamic tracts and spinal trigeminal nucleus. The clinical manifestations include dysphagia, nausea, vomiting, ataxia, Horner's syndrome, ipsilateral loss of pain and temperature over the face and contralateral loss over the trunk and body etc. Manifestations depend on the site of lesion. The most common etiology of Wallenberg syndrome being atherosclerotic occlusion of the arteries followed by secondary thrombosis. However, prothrombotic states rarely present as lateral medullary syndrome. Therefore, we report two cases of Wallenberg syndrome where patients were found to have hyperhomocysteinemia as a risk factor for the prothrombotic state.
Keywords: Hyperhomocysteinemia, posterior inferior cerebellar artery, Wallenberg syndrome
|How to cite this article:|
Chabbra P, Gupta N, Garg A, Khurana S. Hyperhomocysteinemia presenting as Wallenberg syndrome: A report of two cases and review of literature. Med J DY Patil Univ 2015;8:103-5
|How to cite this URL:|
Chabbra P, Gupta N, Garg A, Khurana S. Hyperhomocysteinemia presenting as Wallenberg syndrome: A report of two cases and review of literature. Med J DY Patil Univ [serial online] 2015 [cited 2020 Jul 12];8:103-5. Available from: http://www.mjdrdypu.org/text.asp?2015/8/1/103/148869
| Introduction|| |
The classical risk factors described for stroke include diabetes, hypertension and cardiac disease. But these risk factors are most of the time not found in patients with young stroke. This has led people to search for novel risk factors like hyperhomocysteinemia which has been at times decribed as a modifiable risk factor. ,, Hyperhomocysteinemia generally presents as vascular thrombotic events as stroke, coronary artery disease etc. However, to our best of knowledge hyperhomocystinemia presenting as Wallenberg has not been described in literature. We present to you two such cases.
| Case Reports|| |
The first case is about a 30-year-old male who admitted with the complaints of sudden onset dizziness, dysphagia both to solids and liquids, dysarthria and swaying to left while walking for past 2 days. There was no history of nausea, vomiting, seizure, focal weakness, sensory complaints, difficulty in hearing, drooling of saliva, facial deviation, diplopia or dimness of vision. There was no history of smoking or addiction to any other drug or substance. There was no history of trauma, fever or self-remitting similar episodes in the past. On examination, patient had stable vitals with a blood pressure of 130/80 mm Hg and pulse rate of 94/min. Neurological examination revealed normal higher mental functions, cranial nerve deficit in the form of absent gag reflex on the left side. Rest of the cranial nerve functions were intact. Motor and sensory examination was normal. There was impaired finger nose test on the left side and patient swayed to the left while walking. A jerky horizontal nystagmus was present with fast component to the right side. Romberg's couldn't be tested as the patient had severe ataxia. Rest of the systemic examination was normal. Contrast enhanced magnetic resonance imaging (CEMRI) of the brain [Figure 1]a and b revealed acute infarct left lateral medulla, left cerebellar hemisphere and vermis in the territory of the posterior inferior cerebellar artery (PICA) confirming the diagnosis of Wallenberg syndrome. Other investigations revealed normal hemogram, erythrocyte sedimentation rate (ESR) was 21, C-reactive protein (CRP) was negative. Electrolytes, renal function tests, fasting and postprandial blood sugars were normal. Two dimensional echocardiography (2D ECHO) was also inconclusive. Anti-nuclear antibody, anti neutrophilic cytoplasmic antibody and anti-phospholipid antibody syndrome (APLA) work-up were negative. Factor V Leiden, prothrombin gene and methylenetetrahydrofolate reductase (MTHFR) mutations were not detected. The levels of antithrombin 3 and protein C and S were within normal limits. Serum homocysteine levels were 26.9 μmol/l. Serum folate levels were 5.04 ng/ml and vitamin B12 levels were 181 pg/ml.
|Figure 1: (a and b) Case 1 — contrast enhanced magnetic resonance imaging|
Click here to view
A possibility of hyperhomocysteinemia was kept and patient was given intravenous vitamin B12, mannitol, oral aspirin and statins. He improved and was discharged with an advice to get monthly vitamin B12 injections. Currently, after 2 months of follow-up, patient has recovered with a minimal residual deficit.
A 32-year-old male admitted with complains of sudden onset dizziness for 1 day associated with left sided swaying, intense nausea, vomiting, nasal regurgitation, dysphagia and slurring of speech. There was no history of fever, trauma, seizures, visual deficits, diplopia, loss of taste sensation hearing loss or any history of smoking or substance abuse. On examination, patient had normal higher mental functions. The speech was slurred, Grade 3 nystagmus was present with fast component to the left, however the movements were normal in all the cardinal positions of gaze. The uvula was deviated to the right side. Rest of the cranial nerves were normal. Power was 5/5 in all four limbs and sensory examination was normal. Cerebellar function tests were abnormal on the left side as evident by dysdiadochokinesia, a positive heel shin and finger nose test on the left side. The patient swayed to the left side while walking. There was no Horner's syndrome or facial hemianesthesia. Investigations revealed hemoglobin of 13.6 with and mean corpuscular volume of 101.7 fl. ESR was 56 and CRP was negative. CEMRI [Figure 2]a and b of the brain revealed acute left PICA territory infarct involving the left anterolateral aspect of medulla, posteroinferior aspect of left cerebellar hemisphere and left cerebellar tonsil confirming the diagnosis of lateral medullary (Wallenberg syndrome). Hypercoagulability work-up was negative including APLA w/u, factor V Leiden, antithrombin 3, protein C, protein S, MTHFR mutation and prothrombin gene mutation but serum homocysteine levels were elevated 32 μmol/l. Serum vitamin B12 and folate levels were within normal limits. Lipid profile, fasting and post prandial sugars were normal along with a normal 2D ECHO. A diagnosis of hyperhomocysteinimia was considered and patient was managed conservatively with antiplatelets, statins, oral vitamin B12, folate, Ryle's tube feeding and was discharged. Following discharge patient gradually improved and is currently doing fine with no residual deficit.
|Figure 2: (a and b) Case 2 — contrast enhanced magnetic resonance imaging|
Click here to view
| Discussion|| |
Wallenberg syndrome is one of the most frequently recognized clinical presentation of brain stem infarction. The most common etiology described for this condition is atherothrombotic occlusion of the posterior circulation vessels. The most common symptom varies from dysphonia  in one series to ataxia in other one.  Arterial hypertension is the most common risk factor as described by Rigueiro-Veloso et al.  Lateral medullary infarction in association with hyperhomocysteinemia has never been described. The classical risk factors described for stroke including diabetes, hypertension and cardiac disease are most of the time not found in patients with young stroke. This has led people to search for novel risk factors like hyperhomocysteinemia which has been at times described as a modifiable risk factor. ,, Elevated levels of homocysteine possibly leads to damage of endothelium and acceleration of atherosclerosis providing a fertile soil for stroke. Raised levels of serum homocysteine can result from mutations, vitamin deficiencies, renal dysfunction and drugs like phenytoin, methotrexate, etc. The association between hyperhomocysteinemia and ischemic stroke is biologically plausible, but multiple clinical trials fail to demonstrate any causal association. Many trials have shown that supplementing vitamins (folate, B12 and pyridoxine) in patients who have an ischemic stroke and hyperhomocysteinemia doesn't affect the outcome as well as there is no reduction of risk for a recurrent stroke. ,
| Conclusion|| |
Hyperhomocysteinemia should always be excluded in patients with Wallenberg syndrome especially in young individuals and with no underlying comorbidities. Whether raised serum homocysteine has got a direct cause and effect relationship is difficult to comment at the moment. However based on the clinical profile of our patients and the available evidence probably, we need to accept that further studies are required in this field. Hence, we report these two cases to create awareness among physicians dealing with such cases.
| References|| |
Rigueiro-Veloso MT, Pego-Reigosa R, Brañas-Fernández F, Martínez-Vázquez F, Cortés-Laíño JA. Wallenberg syndrome: A review of 25 cases. Rev Neurol 1997;25:1561-4.
Sacco RL, Freddo L, Bello JA, Odel JG, Onesti ST, Mohr JP. Wallenberg's lateral medullary syndrome. Clinical-magnetic resonance imaging correlations. Arch Neurol 1993;50:609-14.
Brattstrom LE, Hardebo JE, Hultberg BL. Moderate homocysteinemia - A possible risk factor for arteriosclerotic cerebrovascular disease. Stroke 1984;15:1012-6.
Brattström L, Lindgren A. Hyperhomocysteinemia as a risk factor for stroke. Neurol Res 1992;14:81-4.
Brattström L, Lindgren A, Israelsson B, Malinow MR, Norrving B, Upson B, et al
. Hyperhomocysteinaemia in stroke: Prevalence, cause, and relationships to type of stroke and stroke risk factors. Eur J Clin Invest 1992;22:214-21.
Viswanathan A, Raj S, Greenberg SM, Stampfer M, Campbell S, Hyman BT, et al
. Plasma Abeta, homocysteine, and cognition: The Vitamin Intervention for Stroke Prevention (VISP) trial. Neurology 2009;72:268-72.
Cavalieri M, Schmidt R, Chen C, Mok V, de Freitas GR, Song S, et al
. B vitamins and magnetic resonance imaging-detected ischemic brain lesions in patients with recent transient ischemic attack or stroke: The VITAmins TO Prevent Stroke (VITATOPS) MRI-substudy. Stroke 2012;43:3266-70.
[Figure 1], [Figure 2]