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Home , Monomelic amyotrophy, Monoplegia

CHAPTER Approach to a Patient with Hemiplegia and Monoplegia

27 Sudhir Kumar, Subhash Kaul

INTRODUCTION 4. Injury to multiple cervical nerve roots. Monoplegia and hemiplegia are common neurological 5. Functional or psychogenic. symptoms in patients presenting to the emergency department as well as outpatient department. Insidious onset, gradually progressive monoplegia affecting lower limb can be caused by the following Monoplegia refers to weakness of one limb (either arm or conditions: leg) and hemiplegia refers to weakness of one arm and leg on the same side of body (either left or right side). 1. Tumor of the contralateral frontal lobe. There are a variety of underlying causes for monoplegia 2. Tumor of at thoracic or lumbar level. and hemiplegia. The causes differ in different age groups. 3. Chronic infection of brain (frontal lobe) or spinal The causes also differ depending on the onset, progression cord (thoracic or lumbar level), such as tuberculous. and duration of weakness. Therefore, one needs to adopt a systematic approach during history taking and 4. Lumbosacral-, due to diabetes mellitus. examination in order to arrive at the correct diagnosis. Insidious onset, gradually progressive monoplegia, Appropriate investigations after these would confirm the affecting upper limb, can be caused by one of the following diagnosis. conditions: The aim of this chapter is to systematically look at the 1. Tumor of the contralateral parietal lobe. differential diagnosis of monoplegia and hemiplegia and outline the approach needed to pinpoint the exact 2. Compressive lesion (tumor, large disc, etc) in underlying cause. cervical cord region. 3. Chronic infection of the brain (parietal lobe) or APPROACH TO THE DIAGNOSIS OF MONOPLEGIA spinal cord (cervical region), such as tuberculous. Causes of Monoplegia The causes can be classified on the basis of onset- acute 4. Tumor of the brachial plexus. or chronic; and involvement of the limb (lower or upper). Causes of hemiplegia Acute onset monoplegia affecting the lower limb can be Acute onset hemiplegia can be caused by one of the caused by one of the following conditions: following conditions: 1. - affecting anterior cerebral artery territory. 1. Ischemic or hemorrhagic stroke, affecting contralateral cerebral hemisphere, internal capsule, 2. Cerebral venous sinus thrombosis, affecting brainstem or ipsilateral upper cervical cord. superior sagittal sinus. 2. Cerebral venous sinus thrombosis with venous 3. Trauma-head injury, with contusion in the frontal of contralateral cerebral hemisphere. lobe. 3. Acute central infection, such 4. Infection, such as granuloma affecting frontal lobe. as or , brain , 5. Trauma to the lumbo-sacral plexus, diabetic lumbo- granulomatous infections. sacral plexopathy. 4. Head injury, causing contusion/ in the 6. Functional or psychogenic. contralateral cerebral hemisphere, internal capsule, basal ganglia, or brainstem. Acute onset monoplegia affecting upper limb can be caused by the following conditions: 5. Bleeding into a brain tumor on the contralateral side. 1. Stroke, affecting superior division of contralateral middle cerebral artery territory, affecting parietal 6. Demyelinating illness, such as ADEM (acute lobe, or unpaired anterior cerebral artery. disseminated ) or MS (). 2. Head injury, with contusion in the parietal lobe. 7. Todd’s . 3. Trauma to the brachial plexus. 142 8. Metabolic derangements, such as hypoglycemia, The clue towards a likely vascular cause is the sudden hyperglycemia or hyponatremia. onset of weakness, and the exact time of onset of weakness can be obtained from the history. The exception 9. Functional or psychogenic. includes “wake up ”, where the patient goes to Insidious onset, gradually progressive hemiplegia can be sleep without any deficits and wakes up with a new onset caused by one of the following conditions: weakness of the leg. 1. Brain tumor, affecting cerebral hemisphere, internal The diagnosis of stroke can be confirmed by doing a capsule, basal ganglia or brainstem. computerized tomography (CT) scan. Hemorrhage or bleeding is easily picked up on the CT scan. Acute 2. Tumor of the spinal cord in cervical region. infarction may be missed on the CT scan during initial 3. Chronic infections of the brain, such as tuberculosis, few hours. Therefore, magnetic resonance imaging (MRI) hydatid cysts, etc. with diffusion-weighted imaging is the modality of choice 4. Mill’s hemiplegic variant of for diagnosis of acute ischemic stroke causing isolated [4] (MND). monoparesis.

NEUROLOGY Above, we have seen the list of differential diagnosis for Acute onset weakness of contralateral leg can also acute onset as well as insidious onset, gradually progressive occur with cerebral venous sinus thrombosis (CVST) conditions causing monoplegia and hemiplegia. The with venous infarction affecting medial frontal lobe. list mentions the most common causes encountered in Weakness of contralateral leg can also rarely occur due routine clinical practice, and rare conditions have not to granulomatous infections affecting the medial frontal been listed. Still, there are a lot of conditions/diseases lobe and head injury causing contusion/hemorrhage of [5] to be considered while evaluating a case. Therefore, it is the . important to tailor the history and clinical examination to In peripheral nervous system, involvement of lumbosacral arrive at the correct diagnosis. A thorough history taking plexus (lumbo-sacral plexopathy) is the commonest cause and examination would also lead us to order appropriate of acute onset unilateral lower limb weakness. The most investigations, in order to clinch the diagnosis. characteristic feature of lumbo-sacral plexopathy is the In the subsequent sections, we would discuss in detail presence of pain along with weakness of the lower limb. about the approach to monoplegia and hemiplegia. For Pain may be severe and poorly localized. It is described ease of discussion, we would separately consider the as lancinating, aching or burning pain. It may involve acute onset and chronic conditions. thigh, leg and gluteal regions. There is predominant involvement of proximal group of muscles, as compared Acute onset monoplegia affecting lower limb to the distal group of muscles. In addition, there may be In this section, we would consider the conditions that loss of sensations too. Weight loss is noted in most patients. cause acute onset weakness of the lower limb, evolving Clinical examination would confirm the weakness of over hours to days. The site of lesion in a patient with proximal group of muscles. If patients present after a few lower limb weakness can vary from peripheral to central weeks, there may be wasting of thigh and leg muscles. nervous system. Knee jerk is absent. Ankle jerk may be present, however it may be sluggish or absent, if the patient has associated Lesions in the central nervous system more often cause . The diagnosis of lumbo-sacral acute onset monoplegia affecting the lower limb. Among plexopathy can be confirmed by nerve conduction these, the most common are the vascular syndromes. Leg- studies and needle . A clinical clue for predominant weakness with stroke is due to contralateral a plexus lesion (as against multiple peripheral nerves anterior cerebral artery (ACA) infarction in only 25% of and nerve roots) is the motor and sensory involvement cases. More often, it is related to lesions in the contralateral in the distribution of two or more peripheral nerves and corona radiata or internal capsule, in the territory of the two or more nerve root territories in the same limb. The anterior choroidal artery or perforators (30%), or in the common causes for acute onset lumbo-sacral plexopathy brainstem (25%) and can occur with lesions in the middle are diabetes mellitus (also called as Bruns Garland cerebral artery territory or with thalamic hemorrhage.[1] syndrome), viral infections, hemorrhage, vasculitis and Regarding lesions of the medial aspect of the frontal lobe, trauma. In diabetic lumbo-sacral plexopathy, both lower those restricted to precentral gyrus of the paracentral limbs may be affected, however, one side is more affected lobule cause contralateral leg weakness. In rare cases, than the other side. lacunar infarction of the corona radiata can cause ipsilateral weakness of the leg.[2] This can happen due to Acute onset monoplegia affecting upper limb anomalous pyramidal fibers with ipsilateral innervation Lesions in the central as well as peripheral nervous system or due to reorganization of pyramidal fibers due to old can cause acute onset monoplegia affecting upper limb. stroke. Ipsilateral pure motor monoparesis of the leg can also rarely occur with lateral medullary infarction. The most common cause of acute onset isolated arm [3] This occurs due to involvement of corticospinal tract weakness on one side is stroke. However, it should fibers innervating the lower limb caudal to pyramidal be noted that it is uncommon for stroke to present decussation. with isolated arm weakness without associated face CHAPTER 27 143 An [9] The cause found was to be tuberculous In this condition, patients complain of However, we need to suspect a spinal [10] [11] [8] , syringomyelia, as she had a past history of TBM and had that. anti-tuberculous therapy for received Among the peripheral nervous system causes, the most common cause for unilateral lumbo-sacral leg plexopathy. The clinical features of lumbo- weakness would be sacral plexopathy have already been The discussed underlying above. etiology for slowly progressive lumbo- sacral plexopathy could be metastases, neurofibroma & sheath tumors, infections and diabetes mellitus. nerve shoulder and arm pain, and there is weakness of and forearm muscles. hand Another cause to be considered in a patient presenting cord compression in even the absence of sensory as level, 23% of cases may present with unilateral weakness. Slowly progressive monoplegia affecting upper limb Slowly progressive The causes for slowly upper progressive limb would be weakness similar to that affecting of lower limb. This would include spinal cord tumors affecting the cervical cord region, and tumors affectingbrain. theSimilarly, infections parietal such as lobetuberculosis of of these areas could also result in unilateral arm weakness. involvement causes, system nervous peripheral the Among of brachial plexus would present weakness. with unilateral Causes arm plexopathy include of tumors, metastases, infections, slowly Diabetes etc. could also cause progressive brachial plexopathy, the involvement of lumbo-sacral plexus though is more common brachial with diabetes. evaluating evaluating such a case in order to correctly localize and diagnose. causes common the are cord spinal or brain the of Tumors limb. lower of monoparesis isolated progressive slowly of often which leg, one of weakness with presents person The months, to weeks few a over and foot, the in distally starts the proximal spreads group to of involve muscles. This is because the lower limb fibers are located laterally in the may pain radicular and pain back of Presence cord. spinal tumor. a probably most lesion, cord spinal a towards point Examination, in addition to motor weakness, may reveal loss of sensations in the affected leg. Bladder symptoms are unusual with unilateral leg weakness. Diagnosis almost delayed always in a case of is neoplastic spinal cord compression, when the patient presents with unilateral leg weakness. MRI of the spine with contrast is valuable in confirming the diagnosis of spinal cord tumor. The common spinal (benign schwannoma & meningioma include tumors cord tumors). tumors) and metastases & glioma (malignant Tumors of the brain affecting precentral gyrus,frontal medial lobe and paracentral lobule monoparesis of contralateral leg. may present with Tuberculosis of spine and tuberculous meningitis (TBM) can also present with subacute or chronic one weakness leg. A of case of 37-year old woman is reported where she presented with spastic weakness of right leg years duration. of six

[6]

[7] Slowly progressive monoplegia affecting lower limb monoplegia affecting lower Slowly progressive A variety of conditions can cause insidious onset, slowly progressive weakness of one causing such leg. isolated progressive The weakness of site one can leg extend of from brain, lesion spinal cord plexus. Therefore, a to systematic approach is needed while nerve roots and or leg weakness. Stroke presenting monoparesis may be misdiagnosed as a peripheral with nerve isolated arm disorder, because of absence of pyramidal tract signs or of the the speech, involvement limbs. face Distal or lower arm monoparesis is an unusual form of cortical infarct, which occurs in the parietal lobe or central sulcus region, stroke cases. less than 1% of comprising Again, the acute onset is the key to suspicion of stroke. The diagnosis can be confirmed by imaging on MRI brain. diffusion-weighted Acute onset monoplegia of upper limb The most due to peripheral nervous system involvement. can also occur common cause in this category is Brachial brachial plexopathy. plexus lesions that can weakness include injury/trauma, infections, hemorrhage, result in acute onset etc. The classical features include arm and shoulder pain, weakness and sensory disturbances. proximal Weakness group of affects muscles more than the distal group. of muscles. Deep tendon Most wasting patients also have reflexes of the affected upper limb A in clinical the involvement distribution absent. of more may be sluggish or than two spinal nerves and more nerves than is two peripheral a strong indicator The of diagnosis of brachial brachial plexopathy plexus can lesion. be confirmed by doing nerve conduction stimulation studies and with needle Erb’s in EMG. point diagnosing MRI brachial is hyperintense signal on also plexus MRI suggestive of valuable inflammation lesion. zoster varicella by caused neuritis brachial in seen be may An abnormal virus. While evaluating While a evaluating case of acute onset of weakness leg or arm, we must also consider a diagnosis of psychogenic monoplegia Psychogenic same. the for basis functional or is common in younger people, and is more common related factors in stress reveal may history Detailed women. to personal relationships, job or studies in individual. the affected Psychogenic and weakness illness) towards attention (deriving gain may primary with be associated secondary gain (avoidance of stress factor). Examination of a patient with psychogenic monoplegia inconsistent may findings. reveal Power of change, the affected when repeatedly tested. limb may Also, if a person psychogenic weakness of leg were made to with stand by self, she would avoid injury while falling. Hoover’s very important sign while is examining a person suspected to have psychogenic leg weakness. Palm of the examiner’s and leg; normal patient’s of heel the under placed is hand with person a In leg. weak the raise to asked is patient the to due felt be would palm the on pressure weakness, true downward movement of the normal leg, however, in a person with psychogenic weakness, no such mechanical pressure is felt on the palm. 144 with slowly progressive weakness of one limb (upper In the initial period after stroke, or lower limb) associated with wasting is monomelic signs are absent. There is hypotonia of affected limbs .12 The characteristic clinical features and deep tendon reflexes are absent. This state is called are insidious onset in the second and third decades, as cerebral shock. This state should not be confused with male preponderance, sporadic occurrence, wasting a lesion. Presence of aphasia, visual and weakness confined to one limb, and absence of field defects, cortical sensory loss and extensor plantar involvement of the cranial nerves, cerebrum, brain stem, response may help in localizing the lesion to cerebral and sensory system. The electromyographic features, cortex. The typical upper motor neuron signs develop along with histologic features of neurogenic atrophy, after a few days of stroke onset. are suggestive of an anterior horn cell lesion. The slow A note should be made of the cranial nerve involvement. progression of illness for two to four years followed by a Cranial nerve involvement on the same side of hemiplegia stationary phase is observed. There is no clinical evidence localizes the lesion to the or internal of involvement of the other three limbs even in patients capsule. Whereas, involvement of cranial nerves on the with long-standing illness of ten to 15 years’ duration. side opposite to that of hemiplegia (crossed hemiplegia), Monomelic amyotrophy affecting upper limb is also localizes the lesion to the brainstem.[14] In brainstem, the

NEUROLOGY called as Hirayama disease. MRI cervical spine in these localization depends on the cranial nerve involvement. cases show asymmetrical lower cervical cord atrophy in Third cranial nerve involvement along with contralateral about half the cases.[13] hemiplegia localizes the lesion to the midbrain (Weber’s Acute onset hemiplegia syndrome). Involvement of sixth and seventh cranial Acute onset hemiplegia is among the commonest nerves along with contralateral hemiplegia localizes presentations in the emergency department. The the lesion to pons. Ipsilateral hypoglossal palsy and underlying cause of acute hemiplegia can be varied, contralateral hemiplegia localizes the lesion to medial however, brain stroke remains the commonest cause. medulla.[15] Also, it is of utmost importance to make a quick diagnosis Acute onset hemiplegia may also occur due to spinal cord of acute ischemic stroke, as the only approved therapy small in the upper cervical cord. Hemiplegia for acute stroke (thrombolytic therapy with tissue in these cases is on the side of lesion. Sensory examination plasminogen activator) can be given only within the first may also reveal ipsilateral loss of joint position and four and half hours after stroke onset. vibratory sensations, and pain and temperature The most important consideration in the diagnosis of impairment on the contralateral side.[16] The artery stroke is the abruptness of onset of symptoms. The affected is usually anterior spinal artery. typical history of a patient with stroke could be as per In a patient suspected to have stroke, MRI brain or the described case here: “Mr KS went for a morning walk spinal cord (as per the clinical findings) is the imaging as usual and returned in half an hour. He sat down in modality of choice for confirming the diagnosis. Diffusion balcony reading a newspaper and having a cup of tea. All weighted sequences are the most preferred and valuable of a sudden, the newspaper fell off his right hand, and he in detecting acute infarcts. could not hold the cup of tea. He slumped off the chair to the ground. He could not use his right hand or leg. He Bleeding into a tumor in brain is another cause of acute tried calling for his wife but could not.” This is the typical hemiplegia. History of for a few weeks to history of a patient with stroke in left middle cerebral months may point towards a neoplastic etiology. The artery (MCA) territory. In fact, the commonest site of tumor may be located in cerebral cortex, basal ganglia, arterial occlusion in a patient with hemiplegia is MCA thalamus or brainstem. CT or MRI scan of brain would territory. It is also important to enquire about the history confirm the diagnosis. of transient ischemic attacks (TIAs), as several patients In addition, we also need to consider demyelinating would have had one or more TIA in previous 30 days. illnesses and infections as the alternative possible causes The risk factors for stroke such as diabetes, hypertension, for acute onset hemiplegia. Among demyelinating dyslipidemia, hyperhomocystinemia, cardiac disease and illnesses, acute disseminated encephalomyelitis (ADEM) smoking should also be looked into. An enquiry about is more common. ADEM is known to present as acute any accompanying symptoms should also be made. For onset hemiplegia, and may mimic stroke.[17] Patients example, presence of aphasia localizes the site of occlusion with ADEM are relatively younger & healthy, and may to left MCA territory. give a history of fever two weeks prior to the onset of When upper and lower limbs are equally affected, it is hemiplegia. MRI brain is helpful in differentiating ADEM called as dense hemiplegia, and is typical of infarcts in from MS and brain stroke. internal capsule. Motor power is usually grade 0 or 1. On In our country, cerebral venous sinus thrombosis (CVST) the other hand, unequal involvement of limbs point to a is another important diagnostic consideration in patients lesion in cerebral cortex. Upper limbs are more affected with acute onset hemiplegia. Almost always, patients than the lower limbs in a MCA territory infarction, complain of headache for a few days preceding the whereas lower limbs are more affected than the upper hemiplegia. Most patients are young and the risk factors limbs in ACA territory infarction. may be present. These include pregnancy, post-partum CHAPTER 27 145

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[25] J Surg Oncol J Surg 2010; 257:1-4. Copeman MC. Presenting symptoms of neoplastic spinal cord compression. Schneider R, Gautier JC. Leg weakness due to stroke. Site of Site stroke. to due weakness Leg JC. Gautier R, Schneider patterns and causes. lesions, weakness 54. M, Shibata Y, Naito M, Asahi Y, Kawana Y, Li A, Taniguchi et al. Case of ipsilateral monoparesis by lacunar infarction: a consideration of pathological mechanism. 2011; 63:177-80. K, Kishida H, S. Tanaka Pure Tsuda motor monoparesis in the leg due to a lateral medullary infarction. 2012; 2012:758482. A. Pure motor monoparesis due to ischemic stroke. Hiraga 2011; 17:301-8. Neurologist Ando K, Maruya J, Kanemaru Y, Nishimaki K, Minakawa T. Pure motor monoparesis of a lower limb due Nerveinjury: case report. Brain 2012; 64:1427-30. to head Castaldo J, Rodgers J, Rae-Grant A, Barbour P, Jenny Diagnosis D. and neuroimaging of acute stroke distal arm monoparesis. producing caused neuritis Brachial M. Chowdhury V, Raman T, Ayoub plexus brachial in changes by diagnosed varicella-zoster by on MRI. J Neurol Dugas AF, Lucas JM, Edlow JA. Diagnosis of spinal cord compression is nontrauma Med Emerg department. Acad patients in the emergency It is important to suspect a diagnosis of tuberculosis, REFERENCES Patients Patients may complain of headache of raised intracranial are and awakening on worse are type. tension associated with projectile vomiting. There may be visual obscuration at the peak of cases headaches. suspected in CT performed be or should contrast MRI with scan brain to confirm the diagnosis. The commonest brain tumors are metastases and gliomas. Other tumors that can cause are lymphoma, meningioma, neurofibroma, etc. The location of tumor can be in cerebral cortex, basal and brainstem. ganglia, thalamus, a in tuberculoma as such infections consider also must We patient with slowly progressive hemiplegia. Hemiplegia may also be an initial manifestation of CNS tuberculosis. if the patient has fever, headache and weight loss as the symptoms. Institution of may anti-tuberculous reverse the treatment also present hemiparesis. with various Neurosarcoidosis neurological including hemiparesis. manifestations, can is affected, withsymptoms. sparing Clinical of sensorycombination of examination lower and autonomic and upper Needle EMG would confirm the diagnosis. motor would neuron signs. reveal a 8. 1. 2. 3. 4. 5. 6. 7. Slowly progressive hemiplegia Slowly progressive In a patient presenting with insidious onset and slowly progressive hemiplegia, brain important tumors consideration. In are a presenting to ER, the series about 25% of them had hemiparesis. on most brain tumors [24] We We should also consider a diagnosis of Mill’s variant of amyotrophic lateral sclerosis in a person presenting with slowly progressive hemiplegia. 9.

[19]

[21] MRI brain shows [20] As expected, MRI of brain [22] In suspected case of TBM, patient may [18] would also be normal. Weakness may Weakness occur after the first or after many of years and does not appear seizure. after every It should be noted that seizure might also setting occur in of the brain stroke, where sinus thrombosis. embolic strokes and venous isit more common with Metabolic derangements should also be (low Hypoglycemia hemiplegia. acute with dealing while kept in mind blood glucose) is a well known, of but hemiplegia. uncommon Patients cause with hypoglycemia can present with acute hemiplegia associated with other signs such as aphasia, which can mimic strokes. complain of sudden vasculitis onset affecting TBM. The commonestartery hemiplegia, affected often in due TBM to is MCA. with Hemiplegia dengue and falciparum malaria. An has infective cause also been should reported be suspected when the patient with hemiplegia to be done should investigations Appropriate fever. a has presenting patient a in infections these exclude or confirm with hemiplegia. Enquiry should be made about any episode cause common most The hemiplegia. of onset the to prior of seizure of hemiplegia after a seizure could Todd’s be paresis may Todd’s occur paresis. after a generalized or partial seizure, however, it is more common after a generalized seizure. The weakness usually however, lasts it may last for for upto 36 a hours in some few cases. hours, period, period, use of hypercoagulable states, hormonal etc. MRI with pills, MR venogram of nephrotic brain would confirm the diagnosis. Hemiplegia in CVST syndrome, usually occurs due to cerebral cortex. hemorrhagic infarctions in the Infections such as herpes encephalitis and meningitis tuberculous (TBM) can hemiplegia also mimicking stroke. rarely In patients cause encephalitis, with sudden herpes acute-onset onset stroke-like hemiparesis been reported. has infarctions. However, administration and hemiplegia the reverses rapidly glucose of correction of glucose and other neurological deficits. Hemiparesis hasreported also in cases been of hyponatremia (low serum sodium) apparently due to central pontine myelinolysis. Functional or psychogenic cause of hemiplegia fairly common. is The typical also scenario is a young woman with hemiplegia, onset acute with hospital the to brought a seemingly no risk factors for stroke. History may reveal stressful mental state. Examination is very important in we When hemiplegia. of cause psychogenic a confirming raise the arm and let it fall, the person Similarly, hurt. getting avoid typically to face the over fall it letting avoids when we make the patient stand and leave, the patient falls “carefully” to avoid getting hurt. While doing these maneuvers, we should be cautious to avoid injury to the Another cause. psychogenic a to due not were it if patient, useful clinical sign is Hoover sign, as described above in the section on monoplegia. Motor-evoked potentials may be done, which are normal. 146 10. Sundaram SS, Vijeratnam D, Mani R, Ginson D, Chauhan 19. Rolak LA, Rutecki P, Ashizawa T, Harati Y. Clinical features AJ. Tuberculous syringomyelia in an HIV-infected patient: of Todd’s post-epileptic . J Neurol Neurosurg a case report. Int J STD AIDS 2012; 23:140-2. Psychiatry 1992; 55:63-4. 11. Katz JS, Saperstein DS, Wolfe G, Nations SP, Alkhersam H, 20. 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