פרק 4 Chapter 4 Disorders of Movement and Posture Flashcards
איש עם אסטרקסיס ביד ימין בלבד, מהו הגורם?
א. דימום בפוטמן
ב. אוטם בגרעין הקדמי של התלמוס
ג. אנצפלופתיה הפטית
ד. פניטואין
תשובה ב. אוטם בגרעין קדמי של התלמוס.
This sign (Asterixis) was first observed in patients with hepatic
encephalopathy but was later noted with hypercapnia,
uremia, and other metabolic and toxic encephalopathies.
Asterixis may also be evoked by phenytoin and other
anticonvulsants, usually indicating that these drugs are
present in excessive concentrations. Similar rapid lapsing
movements of the head or arms sometimes appear during
drowsiness in normal persons (“nodding off”).
Unilateral asterixis occurs in an arm and leg on the
side opposite an anterior thalamic infarction or small
hemorrhage, after stereotaxic thalamotomy; and with an upper midbrain lesion, usually as a transient phenomenon after stroke.
a unilateral asterixis is usually attributable to
an acute thalamic stroke on the contralateral side, but
there was an interesting variety of other localizations
including the frontal lobe (anterior cerebral artery infarction),midbrain, and cerebellum in a few cases each
מטופל התקבל עם רעד אינטנציונלי, ללא תגובה לטיפול תרופתי. איפה תשתיל אלקטרודות?
א. STN
ב. Thalamus postertior
ג. GPi
ג. GPi
טיפול:
טיפי (non alternating beat)
ביותר מ 75% מהמטופלים, תגובה מעולה לאלכוהול בכמות קטנה. אך בחלוף ההשפעה הרעד חוזר ולעיתים בצורה חמורה יותר.
חסמי בטא אדרנרגיים – פרופנולול / אפשר גם מטאפרולול או נאדולול – לוקח מספר ימים עד שבועות ועוזר ל 50-70% מהמטופלים, אולם גורם לתופעות לוויאי – כגון פגיעה בזקפה, ברונוכספאזם ועייפות. מעניין לציין שהזרקה מקומית של חסמי בטא רצפטור לא הפחית את הרעד וכנראה שהמנגנון עובד דרך CNS, וזאת בניגוד למה שצויין לגבי רעד פיסיולוגי. ייתכן הירידה ברעד משנית לירידה ברעד הפיסיולוגי שמתלבש על הרעד האסנציאלי
ברביטורטים גם יכולים לעזור (קו שני לטיפול לאחר חסמי רצפטורי בטא), אך תופעות הלוואי קשות – אטקסיה קלה, טשטוש ועייפות כרונים.
גאבאפנטין, טופיראמאט, מירטאזיפין ומגון רחב של בנזו’ יכולים לעזור ונחשבים לקו שני של טיפול.
אמנטדין הדגים שיפור קל ברעד וניתן להוסיפו לתרופה מהמצויינות לעיל.
אטיפי (alternating beat) –
איטי, אמפליטודה גדולה יותר וקשה לטיפול, בנזו, ברביטורטים ואלכוהול פחות עוזרים במקרה זה
בוטוליניום, יכול לשפר את ברעד באיבר אליו הוזרק, אולם החולשה הנלוות אינה רצויה.
השתלת אלקטרודות בחלק הונטרלי המדיאלי של התלמוס. או לחלק הפנימי של הגלובוס פלידוס.
In resistant cases of essential tremor of the fast or
slow variety, stimulation by electrodes implanted in the
ventral medial nucleus thalamus or the internal segment
of the globus pallidus (of the same type used to treat
Parkinson disease) has produced a durable response
over many years
ברעד אינטנציונלי חד צדדי- איפה הפגיעה?
א. ipsilateral red nucleus
ב. ipsilateral sub thalamic nucleus
ג. ipsilateral Middle Cerebrellar Peduncle
ד. contralateral cerebellum
תשובה ג. ipsilateral middle cerebellar peduncle
a lesion in which of the following produces an ataxic tremor
א. superior cerebellar peduncle
ב. inferior cerebellar peduncle
ג. interpeduncular nucleus
ד. flocular node
- -Lesions involving the superior cerebellar peduncle or the dentate nucleus cause the most severe and enduring cerebellar symptoms, which manifest mostly as ataxia in the ipsilateral limbs.
- -Disorders of stance and gait depend more on vermian than on hemispheral or peduncular involvement.
- -Damage in the inferior cerebellum causes vestibulocerebellar symptoms-namely, dizziness, vertigo,
vomiting, and nystagmus-in varying proportions.
These symptoms often share with disturbances of the
vestibular system the feature of worsening with changes
in head position
Ataxic tremor
This has been produced in monkeys by inactivating the deep cerebellar nuclei or by sectioning the superior cerebellar peduncle or the brachium conjunctivum below its decussation,
מה לא יבדיל בין רעד פרקינסוני לרעד ראשוני?
א. תדר
ב. מיקום הרעד (למשל ראש ומיתרי קול)
ג. מחמיר בסטרס
ד. מופיע במנוחה או בפעולה
ה. ברדיקינזיה נלווית
תשובה ג. מחמיר בסטרס
תשובה ב רעד ראשוני תנחיל חוסמי ביטא
תשובה ב
esstential tremor
כמעט תמיד מתחיל בידיים, כמעט סימטרי לגמרי (אולם ב15% מהחולים מתחיל ביד דומיננטית). הרעד עשוי להיות מוגבל לגפיים עליונות, או לתנודות של הראש מצד לצד. ייתכן רעד של הסנטר בנוסף או בנפרד. במקרים מסוימים יש מעורבות של לסת, שפתיים, לשון, ולארינגס (יגרום קול רועד – voice tremor). לעיתים נדירות הרעד של הראש או הקול מקדים את זה של הידיים. גם הרעד של הראש הוא פוסטורלי ונעלם כשהראש נתמך. הרעד נעלם כשהחולה הולך. גפיים תחתונות לרוב spared או מעורבות באופן מינימלי. חרדה מחמירה רעד ואלכוהול מפחית אותו.
מה הטיפול orthostatic tremor?
א.propanolol
ב. valporate
ג. topomax
ד. phenytoin
תשובה ב. דפלפט
This unusual fast tremor of the legs may devastate gait, it is present only when the patient stands or exerts force with the legs while seated. The tremor ceases upon walking. In reaction to a perception of severe imbalance, which is characteristic of the disorder, the patient assumes a widened and often stiff legged stance.
This is a rare but striking tremor isolated to the legs that is remarkable by its occurrence only during quiet standing and its cessation almost immediately on walking. It is difficult to classify and more relevant to disorders of gait than it is to tremors of other types. The frequency of the tremor has been recorded as approximately 14 to 16 Hz, making it difficult to observe and more easily palpable. Nonetheless, it may produce considerable disability as the patient attempts to stabilize himself in response to the tremulousness. An important accompanying feature is the sensation of severe imbalance, which causes the patient to assume a widened stance while standing; these patients are unable to walk a straight line (tandem gait) The arms are affected little or not at all. Often the first step or two when the patient begins to walk are halting, but thereafter, the gait is entirely normal. Because falls are infrequent, the condition is often attributed to hysteria. Tremulousness is not present when the patient is seated or reclining,
Some cases have responded to the administration of clonazepam, gabapentin, primidone, or sodium valproate alone or in combination but it often proves difficult to treat. A few intractable cases have been treated with an implanted spinal cord stimulator.
תשובה א
טופומקס זה הטיפול בחלק מסוגי הרעד..
תשובה א. palatal myoclonus
מטופל מתקבל עם רעדבחיךבתדר של 3לשניה, לא נעלם בלילה , באיזה מהבאים יהיה הנגע?
א. תלמוס
ב.Inferior olivary nucleus
ג. Middle Cerebellar Peduncle
ד. Nodule\flocullus
חולה עם רעד, ברקע יש לו מחלת לב, אסטמה קשה, בצקת ברגליים ותנועות בלתי רצוניות, איזה תרופה ניתן לתת לו בהתחשב במחלות הרקע?
א. amantadine
ב. propanolol
ג. L-DOPA
ד. Benztropine
תשובה 4. בנזטרופין- mao inhibitor
Amantadine – no because of oedema
Propranolol – no because of asthma
L-Dopa – SE profile too significant
Either MAOb or anti- cholinergic
אטקסיה/דיסטוניה שמחמירה עם אלכוהול, מה הטיפול?
א. קלונקס
ב. פניטואין
ג. טגרטול
תשובה א. קלונקס.
familial paroxysmal choreoathetosis, and periodic dystonia (paroxysmal kinesigenic dyskinesia, familial paroxysmal choreoathetosis, and periodic dystonia)–
יש 3 סוגים מרכזיים
* הראשונה אוטוזימלית דומיננטית – משפיע על ילדים ונוער – התקפית למספר דקות יכולה להופיע אחר בהלה, היפרונטילציה או תנועה פתאומית- מגיב טוב לפניטואין וטגרטול.
* – התקפים של דיסטוניה פרסיסטנטית הנעים בין 5 דקות ל 4 שעות, ייתכן יונילטרלי או בילטרלי,יכול להיגרם עקב צריכת קפה, אלכוהול או עייפות, אך לא מתחיל אחר תנועה פתאומית ולכן השם
NONKINESIGENIC TYPE- אוטוזומלית דומיננטית.
מגיב טוב לקלונקס. חלק מהמטופלים יציגו דיפלופיה וספסטיות ואחרים התייצגו עם infantile convulsions.
* - סוג שלישי דומה לסוג 2 ובעבר חשבו שזו אותה מחלה. מופק ע”י התעמלות ארוכה, מגיב טוב לאצטזולמיד.
there are three main forms of familial paroxysmal
choreoathetosis.
-One, which has an autosomal dominant (less often recessive) pattern of inheritance and a tendency
to affect males, begins in adolescence or earlier.
It is characterized by numerous brief (several minutes) attacks of choreoathetosis provoked by startle, sudden movement, or hyperventilation-hence the title paroxysmal kinesigenic choreoathetosis. There may be many dozens of attacks per day or occasional ones.
This disorder responds well to anticonvulsant medication, particularly to phenytoin and carbamazepine.
-In a second type, such as those originally described by Mount and Reback and subsequently by Lance and by
Plant et al, the attacks take the form of persistent (5 min to 4 h)dystonic spasms and reportedly have been precipitated by the ingestion of alcohol or coffee or by fatigue but not by movement per se (nonkinesigenic type). The attacks may be predominantly one-sided or bilateral. This form of the disease is inherited as an autosomal dominant trait; a few families have displayed diplopia and spasticity and others have shown a familial tendency to infantile convulsions. Each of these types has a different causative gene. Attacks may occur every several days or be separated by years. A favorable response to benzodiazepines (clonazepam) has been reported, even when the drug is given on alternate days.
-A third type, formerly thought to be a variant of the Mount-Reback type mentioned above, is precipitated by
prolonged exercise. In addition to a response to benzodiazepines, it has the unique characteristic of improving with acetazolamide.
תשובה א. carbamazepine
There are three main forms of familial paroxysmal choreoathetosis and dystonia.
One clinical type, autosomal dominant (less often recessive) ‘tendency to affect males, begins in adolescence or earlier and abates later in life. It is characterized by numerous brief (several minutes) attacks of dystonia or choreoathetosis provoked by sudden movement, startle, or hyperventilation—hence the name paroxysmal kinesigenic choreoathetosis. There may be many dozens of attacks per day or occasional ones. This disorder responds well to antiepileptic medication, particularly to phenytoin and carbamazepine. Mutations of PRRT2, the proline-rich transmembrane protein, have been identified as the cause in some families and link the disease to a variety of infantile convulsions
In a second nonkinesigenic type, the attacks take the form of persistent (5 min to 4 h) dystonic spasms and reportedly have been precipitated by the ingestion of alcohol or coffee or by fatigue but not by movement. The attacks may be predominantly one sided or bilateral. Attacks may occur every several days or be separated by years. A favorable response to benzodiazepines (clonazepam) has been reported, even when the drug is given on alternate autosomal dominant trait; a few families have displayed diplopia and spasticity and others have shown a familial tendency to infantile convulsions..
A third type, precipitated by prolonged exercise. In addition to a response to benzodiazepines, it has the unique characteristic of improving with acetazolamide.
More common than these familial dyskinesias are sporadic cases secondary to focal brain lesions such as stroke, trauma, encephalitis, perinatal anoxia, multiple sclerosis, HIV encephalitis or as a result of associated toxoplasmosis, lymphoma; and also generalized metabolic disorders such as hypoparathyroidism, thyrotoxicosis, and particularly, nonketotic hyperosmolarity.
The most severe instances of paroxysmal dyskinesia in our experience have been related to the previously mentioned multiple sclerosis (“tetanoid spasms”), and from the secondary brain lesions of HIV. These patients were relatively unresponsive to medications. Also, it should be recalled that oculogyric crises and other nonepileptic spasms have occurred episodically in patients with postencephalitic parkinsonism; these phenomena are now rarely seen with acute and chronic phenothiazine intoxication and with Niemann-Pick disease (type C).
איזה מהתרופות הבאות לא גורמת לכוריאה?
א. גלולות
ב. הלידול
ג. L- DOPA
ד. פרופנולול
תשובה ד. פרופנולול
Many drugs in addition to the toxic effects of L-dopa and antipsychotic
medications occasionally cause chorea (amphetamines,
cocaine, tricyclic antidepressants, lithium, isoniazid, linezolid).
א. פניטואין
Nystagmus occurs early, loss of smooth extraocular pursuit movements, Diplopia, Ataxia, Chorea, sedation.
Gingival hyperplasia, hirsutism, coarsening of facial features, mild peripheral neuropathy, usually manifested by diminished deep tendon reflexes in the lower extremities.
Long-term use may also result in
abnormalities of vitamin D metabolism, leading to osteomalacia.
Low folate levels and megaloblastic
A skin rash may indicate hypersensitivity of the patient to the drug. Fever
Lymphadenopathy
Hematologic complications are exceedingly rare, although agranulocytosis has been reported in combination with fever and rash.
איזו מהמצבים הבאים אינו גורם לכוריאה?
א. Hyperosmolar hyperglycemia
ב. Hypothyroidism
ג. APLA syndrome
ד. Polycythemia vera
ה. Phenytoin
ב. היפותירואידיזם
תשובה ד. NPH
תשובה ב. גן לגליצין
איזו הפרעה פסיכיאטרית הולכת ביחד עם טורט ?
א.OCD
ב. DEPRESSIVE
ג. GENERALIZED ANXIETY DISORDER
א. OCD
תשובה ב. וולפורט
The alpha2- adrenergic agonists clonidine and guanfacine have been useful in several studies, but not in others. These are not as potent as treatment with neuroleptic drugs, but their side effects are far less severe and they are recommended as the first treatment. The newer drug, guanfacine has the advantage over clonidine of daily dosing and less sedating effect.
The neuroleptics haloperidol, pimozide, sulpiride, and tiapride have proved to be effective therapeutic agents but should be used only in severely affected patients and usually after the adrenergic agents have been tried.
The potent agent tetrabenazine, a drug that depletes monoamines and blocks dopamine receptors, may be useful if high doses can be tolerated.
clonex for isolated tics
תשובה א. מתוקלופרמיד
תשובה 4. גמילה מאלכוהול
מה יגרום להמיבליזמוס?
Ballistic movements are usually unilateral (hemiballismus) and the result of an acute lesion of or near the contralateral subthalamic nucleus or immediately surrounding structures (infarction or hemorrhage, less often a demyelinative or other lesion). Rarely, a transitory form is linked to a subdural hematoma or thalamic or parietal lesion. The flinging movements may be almost continuous or intermittent, occurring several times a minute, and of such dramatic appearance that it is not unusual for them to be regarded as hysterical in nature.
מהו הנוירוטרנסמיטור בין הסטריאטום לפלידום?
GABA
GABA
תשובה ב.
MEIGE SYNDROME
Meige syndrome is a rare neurological movement disorder characterized by involuntary and often forceful contractions of the muscles of the jaw and tongue (oromandibular dystonia) and involuntary muscle spasms and contractions of the muscles around the eyes (blepharospasm).
Lingual, Facial, and Oromandibular Spasms
These special varieties of involuntary movements also
appear in later adult life, with a peak age of onset in
the sixth decade. Women are affected more frequently
than men. The most common type is characterized by
forceful opening of the jaw, retraction of the lips, spasm
of the platysma, and protrusion of the tongue; or the jaw
may be clamped shut and the lips may purse (Fig. 6-3B).
Other patterns include lateral jaw deviation and bruxism.
Common terms for this condition are Meige syndrome,
Difficulty in speaking and swallowing (spastic or spasmodic dysphonia) and blepharospasm are also frequently conjoined, and occasionally patients with these disorders develop torticollis or dystonia of the trunk and limbs.
Treatment
As with the other focal and regional dystonias, substantial success has been obtained with injections of botulinum toxin into the masseter, temporal, and medial pterygoid muscles. High doses of benztropine and related anticholinergic drugs may be helpful, but are not as effective as botulinum toxin treatment. Many other drugs have been used in the treatment of these craniocervical spasms, but none has effected persistent benefit.
לגבי המסיחים אחרים:
1. טורט -בחלק קטן מהמטופלים – הפרעות תנועה נוספות – דיסטוניה, טורטיקוליס, בלפרוספאזם,
3. וויפל -ההתייצגות הנוירולוגית היא לרוב אובדן זיכון איטי ופרוגרסיבי או דמנציה סב-אקוטית\כרונית. התייצגויות פחות נפוצות – אופתלמופלגיה סופרה נוקלארית, אטקסיה צרבלרית (50%), פרכוסים, מיוקלונוס, ניד,
myorhythmia (תנועות מאוד אופייניות אוקולו-מסטיקטוריות, לאדמס זה בכלל נראה כמו מיוקלונוס ריתמי.)
ב10% מהחולים – לומר לא רגיש, אך מאוד ספציפי). המיוריתמיות, כאשר מופיעות, מלוות בשיתוק מבט ורטיקלי ולעיתים הוריזונטלי.
4. Botolinium -As a rule, blurred
vision and diplopia are the initial neural symptoms
תשובה ג.
The most prominently affected muscles are the sternocleidomastoid, levator scapulae, and trapezius. EMG
studies also show sustained or intermittent activity in
the posterior cervical muscles on both sides of the neck.
The levator spasm lifts the affected shoulder slightly, and
tautness in this muscle is sometimes the earliest feature.
תשובה ג. גליצין
Table 4-1
CLINICAL DIFFERENCES BETWEEN CORTICOSPINAL AND EXTRAPYRAMIDAL SYNDROMES
table 4-1
Table 4-2PROPERTIES AND LOCALIZATION OF DOPAMINE RECEPTORS
table 4-2
Table 4-3CLINICOPATHOLOGIC CORRELATIONS OF EXTRAPYRAMIDAL MOVEMENT DISORDERS
Table 4-4DISEASES ASSOCIATED WITH CHOREA
Table 4-5DISEASES ASSOCIATED WITH DYSTONIA (4)
Inherited (8)
Drug induced (3)
Symptomatic (secondary) (10)
Idiopathic focal (6)
Table 4-6MAIN TYPES OF TREMOR (10)
Table 4-7CAUSES OF GENERALIZED AND REGIONAL MYOCLONUS (5)
Epileptic (11)
Myoclonus dementia (8)
Myoclonus ataxia (3)
Metabolic, immune and toxic (10)
Focal and spinal forms (7)
Figure 4-1. Overview of the components of the basal ganglia in coronal view. The main nuclei of the basal ganglia are represented in light brown, as labeled on the right.
Figure 4-2. Diagram of the basal ganglia in the coronal plane, illustrating the main interconnections (see text for details). The pallidothalamic connections are illustrated in Fig. 4-3.
Figure 4-3. Schematic illustration of major efferent and afferent connections of the basal ganglia. The green lines indicate neurons with excitatory effects, whereas the red lines indicate inhibitory influences. (See text for details and Fig. 4-2.) (Reproduced with permission from Kandel ER, Schwartz JH, Jessell TM: Principles of Neural Science, 5th ed. New York: McGraw-Hill, 2013.)
Figure 4-4.
A. Schematic diagram of the cortical–basal ganglia–thalamic circuits showing the main neurotransmitter pathways and their effects. Dopaminergic neurons arising in the pars compacta of the substantia nigra have an excitatory influence on the direct pathway (via D1 receptors) and an inhibitory effect on the indirect pathway (via D2 receptors).
B. In Parkinson disease, hypokinesia is thought to result from reduced dopamine input from the substantia nigra to the striatum. As a result, decreased inhibition of the globus pallidus interna by the direct pathway, and increased excitation of the globus pallidus interna by the indirect pathway, leads to increased inhibitory drive of the thalamus and therefore decreased excitation of the cortex.
Figure 4-4
C. In Huntington disease, there is degeneration of the striatum. For the direct pathway, there is overall a net inhibition of the globus pallidus interna (due to decreased inhibition from the striatum, increased inhibition from the globus pallidus externa,and decreased excitation from the subthalamic nucleus). For the indirect pathway, there is less inhibition of the globus pallidus externa, leading to more inhibition of the subthalamic nucleus, less excitation of globus pallidus interna. In sum, there is less inhibition of the thalamus, and increased excitation of the cortex, leading to hyperkinetic movements.
Figure 4-5.
A. Characteristic dystonic deformities in a young boy with dystonia musculorum deformans.
B. Sporadic instance of severe axial dystonia with onset in adult life.
C. Incapacitating postural deformity in a young man with dystonia.
Figure 4-6. Tremor branch diagram.
Figure 4-7. Types of tremor. In each, the lowest trace is an accelerometric recording from the outstretched hand; the upper two traces are surface EMG from the wrist extensor (upper) and flexor (middle) muscle groups.
A. A physiologic tremor; there is no evidence of synchronization of EMG activity.
B. Essential (familial) tremor; the movements are very regular and EMG bursts occur simultaneously in antagonistic muscle groups.
C. Neuropathic tremor; movements are irregular and EMG bursts vary in timing between the two groups.
D. Parkinsonian (“rest”) tremor; EMG bursts alternate between antagonistic muscle groups. Calibration is 1 s.
Figure 4-8. Dystonic movement disorders.
A. Young man with severe spasmodic retrocollis. Note hypertrophy of sternocleidomastoid muscles.
B. Meige syndrome of severe blepharospasm and facial-cervical dystonia.
C. Characteristic athetoid-dystonic deformities of the hand in a patient with tardive dyskinesia.
