267 Spastic diplegic gait Patients affected by diplegic cerebral palsy have small and shor t legs in contrast to normally developed chest, shoulders, and arms. In spastic diplegia, there is severe spasticity in the legs, minimal spasticity in the arms, and little or no deficit in speaking or swallowing; whereas in double hemiplegia, there is pseudobulbar palsy and more arm weakness than leg weakness Spastic–ataxic gait If, in addition to spasticity, the disease impairs the dorsal columns or cerebellum, as in spinocerebellar degeneration or multiple sclerosis, patients have a wider-based, unsteady gait and take irregular steps Basal ganglia gaits Marche à petits pas (gait with little steps) Elderly patients with small vessel disease due to arte- riosclerosis, appearing as multiple lacunar infarcts in the basal ganglia, develop a characteristic gait with shuffling, short steps, and are unable to lift the feet from the ground. Progress in walking ceases if the patient tries to speak (they are unable to walk and talk or chew gum at the same time) Parkinsonian gait Patients with degeneration of the substantia nigra or neuroleptic medication toxicity rise and walk slowly with shor t s teps, lack any arm swing, turn en bloc like a statue rotating on a pedestal, and have a tremor when at rest, which disappears during intentional movement Festinating gait When patients are pushed after prior warning, they move forward or backward with tiny steps of increas- ing speed and decreasing length, as if chasing the center of gravity, and they may fall over Choreiform gait When patients with Huntington’s or Sydenham’s chorea walk, the play of finger and arm movements increases, or may even appear clearly for the first time. Random missteps mar the evenness of the strides, as the choreiform twitches supervene Spastic–athetoid gait A combination of athetosis and moderate spastic diplegia or double hemiplegia secondary to perinatal hypoxic damage of the basal ganglia and thalamus has the characteristics of spastic gait, associated with slow, writhing movements of fingers and arms, which tend to increase during walking Equinovarus dystonic gait Dystonia may initially manifest in a child as an inter- mittent inturning of the foot that impedes walking, while in later stages dystonic truncal contortions and tortipelvis may cause the trunk to incline strongly for- ward Types of Stance and Gait Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved. Usage subject to terms and conditions of license. 268 Dromedary gait Patients with dystonia musculorum deformans may take giant, uneven strides, exhibiting flexions or rising and falling of the trunk, like the ungainly gait of a dromedary camel Cerebral gaits Elderly patients with severe bilateral cerebral disease secondary to Alzheimer’s disease, multi-infarct dementia, or senility have difficulty in initiating the sequence of movements for rising, standing, and walking. When starting to walk, patients makes several efforts to move the feet, appearing somewhat puzzled—as if searching for lost motor engrams, or the right buttons to press in order to set off Dancing bear gait The effort to progress may only result in stepping on the spot, as if trying to free the feet from thick, sticky mud Apraxic gait When patients do manage to make progress, the feet cling to the floor as if magnetized Psychiatric gaits Astasia – abasia The patient tilts, gyrates, and undulates all over the place, proving unwittingly—by not falling during this marvelous demonstration of agility—that strength, balance, coordination, and sensation must still be in- tact Sexual behavior and biological orientation gaits The gait is characteristic of and diagnostic of the bio- logical and behavioral state of a person’s brain Heterosexual male– female gait Movement Disorders Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved. Usage subject to terms and conditions of license. 269 Neurotrauma Glasgow Coma Scale* Response Score Eye opening Spontaneous 4 To command 3 To pain 2 No response 1 Best motor response Obeys 6 Localizes 5 Withdrawal 4 Flexor 3 Extensor 2 No response 1 Best verbal response Oriented and conversed 5 Confused conversation 4 Inappropriate words 3 Incomprehensible sounds 2 No response 1 *Teasdale G, Jennett B: Assessment of coma and impaired consciousness. A practice scale. Lancet 2: 81– 84, 1974 Pediatric Coma Scale Response Score Eye opening Spontaneous 4 To voice 3 To pain 2 No response 1 Best motor response Flexes / extends 4 Withdraws 3 Hypertonic 2 Flaccid 1 Best verbal response Cries 3 Spontaneous respiration 2 Apneic 1 Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved. Usage subject to terms and conditions of license. 270 The Unconscious Patient Intracranial lesions Cerebrovascular disease – Hemorrhage ț Intracerebral ț Subarachnoid ț Epidural hematoma ț Subdural hematoma – Infarction ț Arterial occlusion ț Venous occlusion – Trauma (closed head injury) – Epilepsy and postictal states – Neoplasm ț Primary ț Metastatic – Brain edema – Infection ț Meningitis ț Encephalitis ț Abscess – Primary neuronal or glial disorders ț Progressive multifocal leukoencephalopathy (PML) ț Creutzfeldt–Jakob disease ț Adrenoleukodystrophy ț Gliomatosis cerebri Toxic and metabolic encephalopathy Exogenous – Sedatives or psycho- tropic drugs ț Ethanol ț Barbiturates ț Opiates ț Tricyclic antidepressants and anticholinergic drugs ț Phenothiazines ț Heroin ț Amphetamines ț LSD, mescaline – Acid poisons ț Methyl alcohol ț Paraldehyde – Other ț Organic phosphates ț Cyanide ț Heavy metals ț Cardiac glycosides ț Steroids (insulin) Endogenous – Hyperglycemia ț Ketotic coma ț Nonketotic coma – Hypoglycemia Endogenous insulin, liver disease, etc. Neurotrauma Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved. Usage subject to terms and conditions of license. 271 – Uremic coma Kidney failure – Hepatic coma Liver failure – CO 2 narcosis Pulmonary failure – Electrolyte distur- bance ț Dehydration ț Drug-induced ț Heat stroke ț Fever – Endocrine ț Pituitary apople xy and necrosis ț Adrenal (Addison’s disease, Cushing’s disease, pheochromocytoma) ț Thyroid (myxedema, thyrotoxicosis) ț Pancreas (diabetes, hypoglycemia) – Systemic illness ț Cancer ț Sepsis ț Porphyria Anoxia Hypoxic Decreased blood P O 2 and O 2 content – Pulmonary disease – Decreased atmos- pheric oxygen Anemic Decreased blood O 2 content, PO 2 normal – CO poisoning – Anemia – Methemoglobinemia Ischemia Decreased cardiac out- put Congestive heart failure – Cardiac arrest – Severe cardiac ar- rhythmias – Aortic stenosis Decreased systemic peri- pheral resistance – Blood loss and hypo- volemic shock – Syncopal attack – Anaphylactic shock Intracranial vessel dis- ease – Increased vascular resistance ț Subarachnoid hemorrhage ț Bacterial meningitis ț Hyperviscosity (polycythemia, sickle-cell anemia) – Widespread small- vessel occlusions ț Subacute bacterial endocarditis ț Disseminated intravascular coagulation (DIC) ț CNS arteritis (systemic lupus erythematosus) ț Fat embolism The Unconscious Patient Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved. Usage subject to terms and conditions of license. 272 Mental illness Conversion hysteria Catatonic stupor Often a manifestation of schizophrenia Dissociative or “fugue” state Severe psychotic de- pression Malingering CNS: central nervous system; DIC: disseminated intravascular coagulation; LSD: lysergic acid diethylamide; PML: progressive multifocal leukoencephalopathy. Metabolic and Psychogenic Coma In unresponsive patients, metabolic disease can be distinguished from psychiatric disease on the basis of differences between the mental state, the motor signs, the breathing pattern, the electroencephalogram (EEG), and the oculovestibular or caloric reflexes. Comatose patients with metabolic disease – Confusion, stupor and coma precede motor signs – The motor signs are usually symmetrical – The EEG is generally very slow – Caloric stimulation elicits either tonic deviation of the eyes or, if the patient is deeply comatose, no response – Seizures are common Psychologically unresponsive patients – The EEG is normal – Caloric stimulation: there is a normal response to caloric irrigation, with nystagmus having a quick phase away from the side of ice-water irrigation; there is little or no tonic deviation of the eyes. Nystagmus is present – Lids close actively – No pathological reflexes are present – Pupils are reactive or dilated (cycloplegics) – Muscle tone is normal or inconsistent EEG: electroencephalogram. Neurotrauma Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved. Usage subject to terms and conditions of license. 273 Metabolic and Structural Coma Metabolic and structural diseases are distinguished from each other by combinations of motor signs and their evolution, and electroencephalo- gram (EEG) changes. Comatose Patients with Met abolic Disease Patients are usually suffering from partial dysfunction affecting many levels of the neuraxis simultaneously, while at the same time the integ- rity of other functions originating at the same level is retained. In general, a suspicion of metabolic disease should be raised if the follow- ing findings are present. Cognitive and behavioral changes (If these represent the earliest or the only signs) Cognition – Poor memory – Disorientation – Language impairment – Inattention – Dyscalculia Behavior – Agitation – Delusions and/or halluci- nations Diffusely abnormal motor signs (Bilateral and symmetrical) Tremor Myoclonus Bilateral asterixis EEG Diffusely, but not focally, slow Acid–base abnormalities Frequent, with hyper ventilation and hypoventilation Pupillary reactions Usually preserved even if the patient is comatose EEG: electroencephalogram. Metabolic and Structural Coma Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved. Usage subject to terms and conditions of license. 274 Comatose Patients with Gross Structural Disease Patients generally have a rostrocaudal deterioration that is characteristic of supratentorial mass lesions, which does not occur in metabolic brain disease, and the anatomical defect is not regionally restricted as it is with subtentorial damage. The clinical signs are certainly helpful, but there is too much overlap to allow the diagnosis to be established by the clinical findings alone. It is not uncommon, for example, for patients with hepatic encephalopathy or hypoglycemia to develop focal motor signs such as hemiparesis or visual field defects, which are characteristic of a structural lesion, whereas patients with multiple brain metastases may develop nothing other than a global alteration of cognitive function. The laboratory screening listed below are therefore essential for ex- cluding structural disease. CT/MRI with enhancement E.g., metastases, infection Lumbar puncture E.g., infection, meningeal carcinomatosis EEG Hematological work-up – Blood cultures E.g., sepsis, septic emboli – Full blood count – Coagulation tests E.g., PT, PTT, FDP – Blood gases Biochemical work-up – Electrolytes E.g., Na, K, Ca, Mg, PO4 – BUN, creatinine, glucose, lactate – Endocrine tests E.g., FSH, T 3 , T 4 , cortisol – Thiamine, folic acid, vita- min B 12 Drug levels E.g., digoxin, anticonvulsants, theophylline, etc. BUN: blood urea nitrogen; CT: computed tomography; EEG: electroencephalogram; FDP: fibrin degradation product; FSH: follicle-stimulating hormone; MRI: magnetic resonance imaging; PT: prothrombin time; PTT: partial thromboplastin time; T 3 : triiodothyronine; T 4 : thyroxine; The patient should be suspected of suffering from structural brain dis- ease, either alone or in combination with metabolic brain disease, if the following findings are present. Neurotrauma Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved. Usage subject to terms and conditions of license. 275 Coma-Like States The basic brain structure that is responsible for arousal is the ascending reticular activating system (ARAS). This system originates in the brain stem reticular formation, and extends to the cortex via the diffuse or nonspecific thalamofrontal projection system. Reticular activation by means of an external stimulus alerts widespread areas of the cortex and subcortex, enabling the patient to be alert and to think clearly, learn ef- fectively, and relate meaningfully to the environment. If there is damage to the extension of the brain stem reticular system in the thalamus or hypothalamus, the full picture of coma will not occur. Since the brain stem portion of the ARAS is intact, reticular activity in- nervates the nuclei of the extraocular nerves, and patients can open their eyes and look about. The cortex, however, is not sufficiently stimu- lated to produce voluntary movement or speech. These patients are in a coma-like state. The characteristics of the coma-like states are presented in the following tables: Coma-Like States Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved. Usage subject to terms and conditions of license. 276 Characteristics of coma-like states Diagnosis Level of conscious- ness Voluntary move- ments Eye responses Speech Muscle tone Reflexes Clinical and patho- logical studies EEG findings Akinetic mutism (deafferen- tation) Patient seemingly awake, but silent and motionless Lack of move- ment; more often, patients move one side or one arm in a stereo- typed fashion in response to noxious stimuli Eyes dart in the direc- tion of moving objects Vocalizing little or not at all. With stimula- tion, can produce normal, short phrases Usually normal; sometimes slight in- crease in legs “Frontal re- lease signs” such as grasp- ing or sucking may be pres- ent. Often dis- play signs of corticospinal track involve- ment, such as hyperreflexia, and a Babinski sign Lesions affect: 1) bi- laterally the frontal re- gion (anterior cingulate gyri); 2) the dien- cephalomesencephalic reticular formation and globus pallidus; 3) the hypothalamus; or 4) the septal area. The most common cause is occlusion of the small vessels entering the brain stem from the tip of the basilar artery. Less commonly due to severe acute hydro- cephalus, and direct compression by tumors EEG shows slow wave abnor- malities Neurotrauma Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved. Usage subject to terms and conditions of license. [...]... signs of raised intracranial pressure, with papilledema, temporal lobe herniation, and coma In very young infants and in the elderly, fever and vomiting may be more prominent than headaches, and the signs of meningitis may be minimal Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved Usage subject to terms and conditions of license 286 Infections of the... of meningitis have been described with HIV-1 infection At the time of seroconversion to HIV-1, most patients develop cerebrospinal fluid (CSF) abnormalities, and a few develop symptoms of headache, meningitis, encephalitis, myelopathy, and plexitis This acute meningitis is clinically indistinguishable from other forms of aseptic meningitis Tsementzis, Differential Diagnosis in Neurology and Neurosurgery. .. cerebritis and abscess formation Nocardia asteroides CNS infection occurs in 0.3% of immunocompromised patients, as in AIDS, resulting in fever, headache, focal neurological deficits, and multiple brain abscesses Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved Usage subject to terms and conditions of license Parasitic and Rickettsial Infections 291 Candida... second most common pathogen involved, in up to 25% of CSF shunt infections) Tsementzis, Differential Diagnosis in Neurology and Neurosurgery © 2000 Thieme All rights reserved Usage subject to terms and conditions of license Acute Bacterial Meningitis Predisposing condition 297 Pathogenic organism – Gram-negative organisms (isolated in 5 – 20% of shunt infections, particularly in infants) – Other pathogens:... to terms and conditions of license Viral Infections 2 89 Slow Viruses Subacute sclerosing panencephalitis (SSPE) SSPE is a chronic measles infection in children between 5 and 15 years and in young adults The brain shows diffuse and widespread inflammation and necrosis in both the gray and white matter The disease leads to severe neurological dysfunction (stage 1, decline in school performance and behavioral... finding, but is not pathognomonic of acute pontine injury Diffuse brain dysfunction and encephalopathy (anoxic coma, or after prolonged status epilepticus) No definite brain stem lesion Ocular dipping (slow downward eye movement, with fast return to mid-position) Brain stem horizontal gaze reflexes are usually intact Pontine hemorrhage, viral encepha- Reverse ocular bobbing (fast-upward eye litis, and. .. Aspergillosis involving the CNS has findings similar to those of mucormycosis CNS aspergillosis may result either from direct extension of nasal cavity and paranasal sinus infection, or more commonly from hematogenous dissemination By direct extension, Aspergillus invades the cavernous sinus and circle of Willis, resulting in angitis, thrombosis, and infarction In hematogenous spread, septic infarction... EEG in several instances essentially isoelectric, but in other cases regained various patterns of rhythm and amplitude; not consistent from one case to the next Clinical and pathological studies Reflexes Muscle tone Speech Eye responses Voluntary Level of conscious- moveness ments Diagnosis Characteristics of coma-like states Coma-Like States 277 Tsementzis, Differential Diagnosis in Neurology and Neurosurgery. .. albicans Candida CNS infection is a manifestation of disseminated disease, and is associated with intravenous drug use, indwelling venous catheters, abdominal surgery, and corticosteroid therapy CNS infection with Candida species often results in scattered intraparenchymal granulomatous microabscesses secondary to arteriolar occlusion Meningitis is a common feature of CNS candidiasis, resulting from invasion... cerebral ischemia), with intact brain stem Rarely seen in posterior fossa hemorrhage Periodic alternating gaze (ping-pong gaze) Roving of the eyes over the full swing of the horizontal plane in oscillating cycles of 2 – 5 seconds Mid- or lower pontine damage Nystagmoid jerking of a single eye, in a horizontal, vertical or rotatory fashion and occasionally bilateral disconjugate vertical and rotatory eye movements . with slow, writhing movements of fingers and arms, which tend to increase during walking Equinovarus dystonic gait Dystonia may initially manifest in a child as an inter- mittent inturning of the. pathological findings in the brain are also non- specific for arboviral infection. The diagnosis of arboviral infec- tions is made serologically (hemagglutination inhibition, neutralizing antibodies, and. grasp- ing or sucking may be pres- ent. Often dis- play signs of corticospinal track involve- ment, such as hyperreflexia, and a Babinski sign Lesions affect: 1) bi- laterally the frontal re- gion