including a large area of abnormal uptake at the base of the
`skull, consistent with metastatic disease. Plain radio-
`graphs dempnstrated extensive erosive lesions at the base
`of the skull. Needle biopsy of the hepatic lesion demon-
`strated large-cell undifferentiated carcinoma with “squa-
`moid” features. After tracheostomy and gastrostomy,
`palliative chemotherapy and radiation therapy were given.
`
`Discussion. The genioglossus, an extrinsic tongue
`muscle, is stronger than the intrinsic tongue mus-
`c l e ~ . ~ When the tongue is forcibly protruded, action
`of the weaker intrinsic tongue muscles may be
`masked, The intrinsic muscles that turn the tip of
`the tongue are the superior and inferior longitudinal
`m u ~ c l e s . ~ . ~ Unilateral contraction of these muscles
`shortens the tongue ipsilaterally, turning the tip to
`that side.
`Afer unilateral denervation, the protruded tongue
`deviates to the weak side. However, the tip of the
`nonprotruded, unilaterally weak tongue can be
`turned to the normal side, but not to the weak side.
`This pattern on protrusion, a sign of unilateral
`extrinsic muscle weakness, has been well described
`and illustrated. The sign of intrinsic muscle weak-
`ness in the unilaterally weak, nonprotruded tongue
`may be known to some experienced clinicians, but
`has not been illustrated in the neurologic literature.
`Finally, a unilaterally weak tongue protrudes the
`
`cheek on the weak side by using the contralateral
`genioglossus muscle, not by using ipsilateral intrinsic
`tongue muscles to turn the tongue into the cheek.
`
`From the Department of Neurology, West Virginia University School of
`Medicine, Morgantown, WV.
`Accepted for publication February 8,1984.
`Address correspondence to Dr. Riggs, Department of Neurology, West
`Virginia University Medical Center, Morgantown, WV 26506.
`
`References
`1. Wartenberg R. Diagnostic tests in neurology: a selection for
`office use. Chicago: The Year Book Publishers, 1953.
`2. Haymaker W. Bing’s local diagnosis in neurological diseases,
`15th ed. St. Louis: C.V. Mosby, 1969.
`3. DeJong RN. The neurologic examination: incorporating the
`fundamentals of neuroanatomy and neurophysiology, 4th ed.
`Hagerstown, M D Harper and Row, 1979.
`4. Wanvick R, Williams PL, eds. Gray’s anatomy, 35th British ed.
`Philadelphia: W.B. Saunders, 1973.
`
`A neurologic rating
`scale (NRS) for use in
`multiple sclerosis
`
`Article abstract-A neurologic rating scale (NRS) has been developed for
`clinical assessment of MS patients. The scale has been tested on 250 MS
`patients. Assignment of the NRS score is based on assessment of each
`component of the neurologic examination and accurately reflects overall
`neurologic function. Clinical exacerbations are evident as significant devia-
`tions from baseline scores. There was close interexaminer correlation, with
`the range of variability no greater than 2.6%. The NRS is a simple, reliable,
`and sensitive scale that can be used with other objective measurements of
`neurologic function, such as neurophysiologic studies, in the clinical assess-
`ment of MS patients.
`NEUROLOGY (Cleveland) 1984;34:1368-1372
`
`Jack C. Sipe, MD; Robert L. Knobler, MD, PhD; Sherry L. Braheny, MD; George P.A. Rice, MD;
`Hillel S. Panitch, MD; and Michael B.A. Oldstone, MD
`
`Multiple sclerosis presents unique and difficult
`problems in the long-term assessment of patients
`because there are neither diagnostic tests nor reliable
`laboratory indicators of disease activity. Changes in
`clinical status have been the principal means for
`evaluating improvement and assessing new forms of
`therapy. Several rating scales have been developed to
`assess neurologic disability and function in attempts
`to quantify changes in clinical
`Refinements
`of these rating scales have also been proposed.6-8 The
`scales are based primarily on activities of daily living
`rather than on the standard neurologic examination,
`
`and they are insensitive to many changes in neu-
`rologic function; some are too elaborate for efficient
`use.
`Increased interest in clinical tria1~’O-l~ demon-
`strates the need for a simple, reliable, sensitive, and
`clinically reproducible scale. We describe a neu-
`rologic rating scale (NRS) that was developed in
`conjunction with a standard neurologic examination
`protocol.
`
`Materials and methods. Patients. We studied 250
`MS patients who fulfilled the conventional diag-
`
`1368 NEUROLOGY 34 October 1984
`
`Hopewell EX1083
`Hopewell v. Merck
`IPR2023-00480
`
`1
`
`

`

`nostic criteria?J4J5 Twenty-four patients with the
`exacerbating-remitting form of MS were enrolled in
`a clinical treatment protocol and were examined at
`least 15 times each year for 3 years. Other patients
`were examined at 3-month intervals or more fre-
`quently during periods of clinical fluctuation.
`Neurologic examination protocol. A neurologic his-
`tory and examination form (figure 1) was completed by
`a neurologist experienced in the evaluation of MS
`patients. Normal neurologic function was graded as
`zero, with 1+, 2+, 3+, and 4+ indicating incre-
`ments of activity (mild, moderate, severe, or max-
`imally increased), and grades of - 1, - 2, - 3, and - 4
`indicating decrements of functional activity (mild,
`moderate, severely reduced, or absent). The grading
`system was applied to mental status, cranial nerves,
`motor system, sensation, and tendon reflexes.
`Neurologic rating scale (NRS). The assignment of
`points in the NRS directly reflects the examiner's
`clinical assessment of each component in the neu-
`rologic examination (table 1). An intact system
`receives the full "normal" point value, with a pro-
`gressive loss in points for mild (-1; l+), moderate
`Severe ( - 3; 3 + ) and maximal ( - 4; 4 + ) deficits are
`(-2; 2+), or severe (- 3, -4; 3+, 4+) involvement.
`scored as severe on the NRS. A category for impor-
`tant subjective symptoms such as bladder, bowel, or
`
`sexual dysfunction was incorporated, because there
`is no simple way to measure central autonomic func-
`tion. The total point distributions for the several
`systems are specifically weighted for common fluc-
`tuating neurologic abnormalities of MS, such as
`visual, motor, sensory, and cerebellar signs. Tendon
`reflexes and Babinski responses, more often present
`than other signs, are less emphasized. Disorders of
`cognition, affect, and mood are also included. The
`final score is obtained by noting the assigned points
`in each of the columns and adding the subtotals. A
`neurologically normal individual would have a score
`of 100 points.
`
`Results. The NRS has been applied to 250 MS
`patients. Serial NRS scores for six MS patients are
`provided (figure 2). The numerical value for each
`patient encounter measures residual neurologic
`function. Clinically significant exacerbations were
`manifested as negative deviations from baseline val-
`ues, and clinical improvement led to an increase on
`the NRS scale (eg, figure 2, patient A). Kurtzke
`Disability Status Scores (DSS)4 provide a correla-
`tion with the status of the patient but were less
`sensitive to clinical changes than were NRS scores
`(figure 2, patients A-F).
`Four neurologists independently scored the NRS
`
`SOYEL L BLADDER: 2
`
`PRESENT MEDS;
`
`SEXUAL FUNCTION;
`
`PERTINENT FLNDIHGS ON GENEPAL E U H I U T I O N :
`
`BP
`
`PULSE
`
`-* " . Y . Y _
`WOO:
`Appropriate-
`Other:
`
`#WOW:
`D i g i t Span: F B-
`P i C t Y I e I :
`i a i a t e -
`5 m!n-
`card#-
`i m d l a t e -
`5 mi"
`
`Verbal:
`
`S e r i a l 7 ' 5
`Similarities:
`appropriate
`~ n a p p i o p i i a t e
`
`Y A L l l N G TIME
`
`+ +
`
`. _ -
`
`I -
`
`Figure 1. Neurologic history and examination form completed by the examining neurologist at each patient encounter.
`The grading convention for all systems is noted at the upper left corner of the form.
`
`October 1984 NEUROLOGY 34 1369
`
`2
`
`

`

`Table 1. Scripps Neurological Rating Scale (NRS) worksheet*
`I
`
`Maximum
`Points
`
`System Examined
`
`Mentation and Mood
`
`Cranial Nerves: Visual Acuity
`Fields, Discs, Pupils
`Eye Movements
`Nystagmus
`
`Lower Cranial Nerves
`
`1
`
`Motor: RU
`LU
`RL
`LL
`
`DTRS: UE
`LE
`
`Babinski: R L (2 ea)
`
`Sensory: RU
`LU
`RL
`LL
`
`Cerebellar: UE
`LE
`
`Gait; Trunk and Balance
`Special Category:
`Bladder/Bowel/Sexual Dysfunction
`Totals
`Neuroloeical Ratine Scale Score
`
`10
`
`21
`
`5
`
`20
`
`8
`
`4
`
`12
`
`10
`
`10
`
`0
`100
`
`Normal
`
`10
`
`5
`6
`5
`5
`
`5
`
`5
`5
`5
`5
`
`4
`4
`
`4
`
`3
`3
`3
`3
`
`5
`5
`
`10
`
`0
`
`I
`
`Degree of Impairment
`Severe
`Mild
`Mod.
`
`7
`
`3
`4
`3
`3
`
`3
`
`3
`3
`3
`3
`
`3
`3
`
`4
`
`1
`2
`1
`1
`
`1
`
`1
`1
`1
`1
`
`1
`1
`
`-
`
`-
`
`2
`2
`2
`2
`
`3
`3
`
`7
`
`1
`1
`1
`1
`
`1
`1
`
`4
`
`0
`
`0
`0
`0
`0
`
`0
`
`0
`0
`0
`0
`
`0
`0
`
`0
`
`0
`0
`0
`0
`
`0
`0
`
`0
`
`-3
`
`-1
`
`10
`-
`
`* Points assigned for each component of the neurologic examination are subtotaled, and points for autonomic dysfunction are subtracted, leaving
`the final (NRS) score.
`
`for five individual patients, using the neurologic his-
`tory and examination form that had been completed
`by an examining physician; the resulting NRS scores
`were in close agreement (table 2), with a range of
`variability less than 2.6%.
`
`Discussion. The MS NRS has been introduced and
`tested as a clinical indicator in the evaluation of
`patients with MS. It provides a rapid summation of
`neurologic function as objectively measured by the
`neurologic examination (figure 1) and, in practical
`terms, provides a convenient quantitative base of
`information (table 1 and figure 2) for neurologic func-
`tions of MS patients who are followed serially.
`NRS scores are more sensitive indicators of clini-
`cal change than the Kurtzke DSS, allowing for rapid
`recording of clinical changes that may not be identi-
`fied in the DSS (figure 2). Isolated new clinical find-
`
`ings during an exacerbation, such as internuclear
`ophthalmoplegia, can make up to a 10-point change
`in the NRS score without altering the DSS. For
`example, patients D and E (figure 2) had 17-point
`and 20-point declines in NRS scores, while DSS
`scores did not change. Similarly, the NRS score of
`patient A (figure 2) revealeda 17-point improvement,
`while the DSS was unchanged. Analyses based on the
`DSS alone may mask important changes in neu-
`rologic function.
`The NRS is not intended to replace the DSS, but
`is a more sensitive, complementary clinical method.
`It is suited for clinical studies with serial observation
`and may be used with other objective measurements
`such as neurophysiologic studies, spinal fluid exami-
`nations, or other laboratory data. The NRS can be
`used in either a prospective or retrospective manner,
`depending on the study design. Because it is simple,
`
`1370 NEUROLOGY 34 October 1984
`
`3
`
`

`

`70m+2
`50 t
`
`I
`12
`
`I
`18
`
`I
`24
`
`#
`
`6
`
`8
`
`40
`30
`0
`90 r
`8 0 t
`
`60
`
`40
`30
`0
`
`0
`
`9Or
`
`70
`
`30 0
`
`60 ::Ic
`80 gT
`
`I
`
`6
`
`I
`
`18
`
`12
`
`,
`
`24
`
`Time lmonthsl
`
`30
`
`0
`
`Table 2. Comparison of Scripps NRS scores by
`four neurologists*
`
`Pt D r . l Dr.2 Dr.3 Dr.4 Mean f SD
`
`
`
`8
`
`96.5 f 1.9
`96
`98
`94
`98
`1
`83.0 IT 2.5
`81
`81
`84
`2
`6
`65.3 f 1.3
`67
`65
`64
`3
`65
`71.0 f 2.6
`70
`72
`68
`4
`74
`53.8 f 1.7
`56
`54
`52
`5
`. 53
`* Each physician independently scored the same neurologic
`examination (previously recorded by another physician) for
`each of five individual patients.
`
`I
`6
`
`4
`
`1
`6
`
`I
`6
`
`I
`
`" \ 6
`Figure 2. Composite
`illustration of the clinical
`course for six patients (A-F).
`The solid line represents the
`NRS score, and arrows
`indicate clinical
`exacerbations of MS. Kurtzke
`DSS scores are indicated
`numerically below the line
`during exacerbations and
`above the NRS line during
`periods of clinical stability or
`improvement.
`
`I
`12
`
`I
`18
`
`I
`24
`
`I
`12
`Time lmonthsl
`
`I
`18
`
`I
`24
`
`From the Departments of Neurology and Immunology (Drs. Sipe, Knobler,
`Braheny, Rice, and Oldstone), Scripps Clinic and Research Foundation, La
`Jolla, CA, and the Department of Neurology (Dr. Panitch), University of
`California, San Francisco, CA. This is publication number 3202 from the
`Department of Immunology, Scripps Clinic and Research Foundation, La
`Jolla, CA.
`This work was supported by National Multiple Sclerosis Society Research
`Grants 1285 and 1307, United States Public Health Service Grant
`"8-12428, and National Institutes of Health Grant RR00833 to the General
`Clinical Research Center at Scripps Clinic and Research Foundation, and
`grants from the Leiper Trust and the McDonald and Hearst Foundations.
`Robert L. Knobler is a recipient of Teacher Investigator Development
`Award, NS-00803, from the National Institute of Neurological and Commu-
`nicative Disorders and Stroke.
`Accepted for publication February 8,1984.
`Address correspondence and reprint requests to Dr. Sipe, Division of Neu-
`rology, Scripps Clinic and Research Foundation, 10666 North Torrey Pines
`Road, La Jolla, CA 92037.
`
`the NRS may also be used to follow the course of
`disease or the effectiveness of treatment in non-
`research patients.
`
`References
`
`Acknowledgments
`
`We would like to thank the staff of the General Clinical
`Research Center and Kaye Smith, RN, for aid in patient care,
`and Miss Gay L. Wilkins for assistance in manuscript prepa-
`ration.
`
`1. Kurtzke JF. On the evaluation of disability in multiple scle-
`rosis. Neurology (Minneap) 1961;11:686-94.
`2. Kurtzke JF. Further notes on disability evaluation in multiple
`sclerosis with scale modifications. Neurology (Minneap)
`1965;15:654-61.
`3. Kurtzke JF. A proposal for a uniform minimal record of dis-
`ability in multiple sclerosis. Acta Neurol Scand [Suppl]
`1981;64:110-29.
`4. Kurtzke JF. A new scale for evaluating disability in multiple
`sclerosis. Neurology (Minneap) 1955;5580-3.
`5. World Health Organization. International classification of
`impairments, disabilities, and handicaps. Geneva: World
`Health Organization, 1980.
`6. Granger CV. Assessment of functional status: a model for
`
`October 1984 NEUROLOGY 34 1371
`
`4
`
`

`

`multiple sclerosis. Acta Neurol Scand [Suppl] 1981;644047.
`7. Kurtzke JF. Rating neurologic impairment in multiple scle-
`rosis: an expanded disability status scale (EDSS). Neurology
`(Cleveland) 1983;33: 1444-52.
`&Mickey MR, Myers LW, Clark 0, Ellison GW. An illness
`severity score for multiple sclerosis. Neurology (Cleveland)
`1983;33(Suppl 2):72.
`9. Poser CM, Paty DW, Scheinberg L, et al. New diagnostic
`criteria for multiple sclerosis: guidelines for research protocols.
`Ann Neurol 1983;13:227-31.
`10. Hauser SL, Dawson DM, Lehrich JR, et al. Intensive immu-
`nosuppression in progressive multiple sclerosis. N Engl J Med
`1983;308 173-80.
`
`11. Fischer BH, Marks M, Reich T. Hyperbaric-oxygen treatment
`of multiple sclerosis. N Engl J Med 1983;308:181-6.
`12. Mertin J, Kremer M, Knight SC, et al. Double-blind controlled
`trial of immunosuppression in the treatment of multiple scle-
`rosis: final report. Lancet 1982;2:351-4.
`13. Aronson SM, Bauer HJ, Brown JR, et al. Therapeutic claims
`in multiple sclerosis. International Federation of Multiple
`Sclerosis Societies, 1982.
`14. Schumacker GA, Beebe GW, Kibler RF, et al. Problems of
`experimental trials of therapy in multiple sclerosis. Ann NY
`Acad Sci 1965;22:552-68.
`15. McDonald WI, Halliday AM. Diagnosis and classification of
`multiple sclerosis. Br Med Bull 1977;33:4-9.
`
`Transient cataplexy
`after removal of a
`craniopharyngioma
`
`studied a patient with cataplexy secondary to a
`Article abstract-We
`surgical lesion that involved the perichiasmal hypothalamus. We believe
`that this lesion interfered with the hypothalamic mechanism for timing
`sleep and wakefulness, whereas the pontine mechanism for generating sleep
`cycles remained relatively intact.
`NEUROLOGY (Cleveland) 1984;34:1372-1375
`
`William J. Schwartz, MD; John W. Stakes, MD; and J. Allan Hobson, MD
`
`Narcoleptic patients (whether falling asleep at
`night or inappropriately during the day) frequently
`begin sleep with a rapid eye movement (REM)
`period. Cataplectic attacks are polygraphically indis-
`tinguishable from REM sleep and often develop into
`full-blown REM sleep episodes.1,2 The etiology is
`unknown but may become better understood by
`study of symptomatic cases that follow anatomically
`discrete structural lesions of the brain, as in the
`following case.
`
`Case report. A 14-year-old girl was admitted in March
`1966 for evaluation of metabolic bone disease; she had
`slipped femoral capital epiphyses at age 12 and an
`atraumatic left tibia1 fracture at age 14. She had developed
`normally, except that she was always below average in
`height. Occasional frontal headaches had appeared at age
`13, and she required glasses for reading. Menstruation had
`not occurred. She weighed 49.8 kg (75th percentile) and
`was 140 cm in height (5th percentile). Pubic hair was
`present, and there was early breast development without
`areolar pigmentation. Examination was normal except for
`diminished visual acuity in the right eye (20/80) and
`bitemporal visual field defects. Skull films revealed an
`enlarged sella turcica (3 cm in maximal dimension), demi-
`neralized posterior clinoid processes, and extensive
`suprasellar calcification. EEG showed paroxysmal bursts
`of bilaterally synchronous high-voltage theta activity with-
`out lateralizing or focal features.
`At surgery, a huge tumor was found to occupy the
`suprasellar region. The entire optic chiasm was forced
`upward to the left, the right optic tract appeared destroyed,
`and the mass extended upward in the midline behind the
`chiasm, pushing the third ventricle upward and backward.
`The tumor was removed completely; pathologic examina-
`tion revealed craniopharyngioma and hypothalamic neu-
`rons. Postoperatively, her visual acuity was 20/500 in the
`
`1372 NEUROLOGY 34 October 1984
`
`right eye and 20/200 in the left eye, and she had a left ho-
`monymous hemianopia.
`She was discharged taking phenytoin. In the next year,
`she required thyroxine, Pitressin Tannate, cyclic estro-
`gens, and hydrocortisone during periods of stress or infec-
`tion. In 1967, she returned to school. Her full-scale I& was
`108.
`Description of sleep disturbance. In October 1968,
`she began to fall asleep in class, sometimes suddenly
`and without warning. In December 1970, she had
`“fainting” spells that lasted 60 to 90 seconds with
`apparent unconsciousness and loss of postural tone.
`These increased in frequency from once weekly to
`several daily by early 1971. In some attacks, she had
`hallucinoid dreams. There was no family history of
`sleep disturbance. Examination was unchanged,
`except for appearance of bilateral optic atrophy; she
`weighed between 55 and 65 kg and was 148 cm in
`height. EEG revealed frequent paroxysmal bursts of
`diffuse bilaterally synchronous high-voltage theta
`activity; spike discharges were occasionally seen not
`confined to the operative area. Her symptoms were
`unchanged after trials of phenytoin, phenobarbital,
`and ethosuximide in varying dosages and combina-
`tions.
`She was readmitted in February and April of 1971.
`In the attacks, she lost postural tone without warn-
`ing. If she were standing, she would gradually fall to
`the floor; if she were in bed, her head would fall back
`onto the pillow. Eyes were closed, but rapid move-
`ments of the globes could be seen beneath the lids.
`Tendon reflexes were not obtained. No movements,
`incontinence, injury, cyanosis, diaphoresis, or
`altered pulse or respiration were seen. She would
`awaken immediately if spoken to or pinched, appar-
`ently aware that she had had a spell. She often
`reported “bad dreams,” incorporating the awakening
`
`5
`
`

`

`A neurologic rating scale (NRS) for use in multiple sclerosis
`Jack C. Sipe, Robert L. Knobler, Sherry L. Braheny, et al.
`1984;34;1368
`Neurology 
`DOI 10.1212/WNL.34.10.1368
`
`This information is current as of October 1, 1984
`
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