28 year old female presenting with chronic progressive asymmetrical weakness of right upper limb and lower limb
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She is a 28 years old, resident of West Bengal and farmer by occupation.The patient presented to the hospital with chief complaints of
Pain and weakness in the lower back, right upper limb and right lower limb since 8 years.
History of presenting illness :
She was apparently alright 8 years ago.
On her usual day as she was drawing water from well, as she bending forward, she experienced pain similar to a muscle pull in her right lower back which relieved on its own in a few minutes. The pain was of sudden onset, dragging in character and progressive in nature. The time frame for the pain to spread has not been specified but the patient reports it to be gradual. The pain spread from her right lower back to her left lower back, then to her right upper limb and her right lower limb. Patient reports the pain spreading laterally to the arm (more intensity on the right arm than the left) and then afterwards spreading to the leg posterio-laterally. The pain aggravated on movement and relieved on taking medication over the counter.
Joint pain : she complained of joint pains on asking her to describe, severity of the pain changed from one joint to another joint at different times. She mentioned the joint pain to be intermittent in severity but it has always been present since the pain at the hip region has started, aggravated on movement, relieved on resting. At night, the patient experiences more pain than in the morning. While in the morning, the patient experiences joint stiffness.
For the past 3 years, the patient experiences an increase in the severity of the pain, because of which she resorted to doing only light household work and farm work. She also describes a change in her gait, due to the pain. The patient also describes reduced sensations in the sole of her foot since three years, and frequent cramping of her right foot, frequent episodes of ?tingling in her right lower limb, more of a dragging sensation.
As for the weakness, she hasn't exactly specified the time frame.
Progression of Pain:-
For the period of first five years, the pain has been increasing gradually after an interval of four to five months. If daily activities are taken into consideration, then the pain increases whenever she does daily chores that involve sitting like cooking, cleaning, washing, lifting heavy bags and also when she gets up in the morning. For lifting heavy bags, she mentions that after thirty minutes of lifting the object... She starts to feel numb. For waking up in the morning, she mentions that when she starts walking around after getting up, the said pain subsides. However if she remains sitting on the bed... The pain gradually increases. The pain reduces when she isn't involved in doing any work.
In the last three years however, the patient describes the pain to have drastically increased, with the pain spreading to the joints as well. The joints involved are the elbow joint, joints at the phalanges, knee joint and hip joint. The pain at the time of examination, as the patient reports, was at the cervical region of the back as well.
She has pain in the proximal and distal interphalangeal joints of right thumb, index and middle fingers, wrist joint and elbow, negative for shoulder bilaterally and thumb, ring and middle finger in the left hand. She also mentions tenderness in her joints.
Weakness:-
She describes it be associated when the pain has started. In the first five years, she has mentioned that she could do chores but with great difficulty especially with regards to sitting. But the main difference here is that in the recent three years... She requires support in performing daily activities such as going to the toilet, eating, sqatting and so on. From what I have observed, she requires support especially at the joint region.
At the time of examination, the patient is able to squat and get up on her own but difficulty in performing lifting the head up with the support of hands lying supine and with arms spread Anteriorly. She also mentions that the severity of the joint pain is less at the moment.
3 years ago, the patient was bitten by a snake ad was rushed to the doctor for treatment. She was given an injection(not known ), due to which she developed a rash over her right arm and forearm, the medication was immediately stopped. The patient suggests that her pain has increased after this incident.
1 year ago the patient visited a doctor, who gave her medication for the pain and suggested to visit a neurologist. The patient consumed the medicines for 1 year, which led to the relief of the pain. On discontinuing the medication the pain increased.
The patient also complains of weakness in her right upper limb, right lower limb and lower back.
The weakness was insidious in onset (began 8 years ago with the pain ) , and progressive in nature (first in the right lower back, then progressed to the right upper and lower limb).
The patient describes an inability to climb stairs, inability to bring food to the mouth from the plate without supporting her right elbow, and difficulty in combing her hair with help of the right hand. (Proximal lower limb and proximal upper limb involvement on the right side).
She also describes difficulty in rolling over the bed, the patient sleeps in one position the entire night. She also has difficulty In raising her neck from the bed while she is lying down, without support. There is presence of flaccid muscles
2 – 3 months ago, the patient experienced giddiness,following intake of a medicine ( name not known - which she is still conuming ) which was sudden in onset, lasted 1-2 minutes, not associated with tinnitus ,aura,or fits. She experienced such episodes every 5 to 6 days.
The patient also complains of headache, at the vertex, sudden in onset, 4-5 episodes per month from the past 2-3 months, that relieve on massage.
No history of swelling, skin changes , rash , difficulty in breathing, fasciculations of the muscles, involuntary movements, changing of speech, spilling of food from the mouth, sweating disturbances or palpitations, fever or vomiting.
*Past history:- The patient is not a known case of diabetes mellitus, hypertension, tuberculosis, asthma, epilepsy.
The patient describes one occasion fall from her bicycle, which led to pain in her right inguinal and lumbar region. The pain subsided on its own.
*Family history:- The patient was born in a non-consanguineous marriage.
The patient’s son experienced one episode of pain and weakness in his lower limbs, which was sudden in onset, did not precede any trauma or fall, non-progressive, which was relived on massage. He was unable to move legs from the bed. Since then he has not had a recurrent episode.
Personal History*
Married
Mixed diet
Adequate quality and quantity of sleep
Normal appetite
Normal bowel and bladder movements
Addictions: consumption of supari since 4 years , 2 - 3 times a day
General examination :
Patient is conscious, coherent & cooperative.
BP - 190/110 mm Hg
After standing for 3 mins
BP - 160/110 mm Hg
Temp - Afebrile
RR - 16 cpm
No signs of Pallor, Icterus, Cyanosis, Clubbing, Generalized lymphadenopathy or edema.
Systemic examination:-
Nervous System Examination
Higher Mental Functions
1. Level of consciousness - Normal (GCS 15/15)
2. Attention - Intact
3. Orientation - to time, place and person - Intact
4. Language - fluency & latency, comprehension, repetition, naming, reading and writing - Intact. Prosody - impaired
5. Memory - immediate recall, recent and remote - Intact
6. Other higher mental functions - general knowledge, abstraction, judgement, insight and reasoning - Intact.
MMSE
29/30 (No cognitive impairment)
CN Examination
1st
Normal (smell of soap).
2nd
Counting fingers at 6mts both eyes normal.
3rd,4th,6th
Pupil size. N N
DLR/CLR. N. N
No pstosis, nystagmus.
5th
Both sensory & motor normal.
Corneal & Conjunctival reflex +.
7th
Nasolabial fold normal.
No deviation of mouth.
Salivation & Lacrimation unaffected.
8th
Rinne's AC>BC.
Weber's - No lateralization.
9 th, 10th &
Palatal movements normal.
No difficulty in swallowing.
Gag reflex present.
11th
Movements of neck in all directions+.
Lifting of shoulders +.
12th
Tone of tongue - Normal.
No wasting, no fibrillations & deviation of tongue.
Tongue tremor+.
Motor examination :
✓Reflexes
Superficial reflexes Right Left
Corneal
Conjunctival
Abdominal
Plantar
Deep tendon reflexes Right Left
Biceps
Triceps
Supinator
Knee
Ankle
Involuntary movements - Resting tremors of Right upper limb , 3-4Hz, high amplitude.
Gait - Reduced arm swing.
Finger tap and toe tap - Normal.
No decrease in speed on repeating the movement continuously.
✓SENSORY SYSTEM Right Left
Pain
Fine-touch
Temperature
Vibration
Medial malleolus
Patella
Elbow
Wrist
Proprioception
Stereognosis
✓CEREBELLUM
Titubation - absent
HINTS
Head Impulse - negative
Nystagmus - negative
Test for skew - negative
Gait Ataxia absent.
Dysarthria absent.
Rebound phenomenon absent.
Intentional tremors - absent.
Pendular knee jerk - absent.
Tandem walking normal.
Coordination tests :-
Dysdiadochokinesia absent
Finger nose test
Heel knee
Rombergs Test -
Axial Skeleton
Inspection -
Palpation -. No spine tenderness.
Movements - Atlanto-occipital - Flexion, extension and lateral flexion normal
Atlanto-axial - Rotation of head normal
Spinal Flexion, Spinal Extension, Lateral Flexion and Rotation are normal
Appendicular
Skeleton -
Upper Limbs (Positive Findings)
Shoulders (both sides)
Inspection - Attitude - Slightly flexed and internally rotated; Contour normal; No edema or erythema
-
Palpation - Mild increase in temperature on both sides
- Range of Movements - Mild Active and Passive limitation of all range of movements (flexion, extension, adduction, abduction, internal rotation and external rotation)
Elbows (both sides) -
-
Inspection - Attitude - mid-flexion;
alignment of elbow and forearm - normal;
Edema + ; No scars or sinuses; no muscle wasting
-
Palpation - All Inspectory findings are confirmed;
Raised temperature +;
Edema +;
Wincing on touch + ;
Fluctuation test + ; 3 point bony relationship intact
-
Range of Movements - Severe pain on active movements of flexion, extension; Mild pain with supination and pronation;
Wrists (both sides) -
-
Inspection - Attitude - Mild extension;
Radial deviation of wrists +;
Diffuse edema +;
Redness +;
-
Palpation - All Inspectory findings confirmed;
Temperature raise +;
Wincing on touch +;
-
Range of Movements - Severely limited and extremely painful active movements of flexion, extension, radial deviation and ulnar deviation.
Hands (both sides) -
-
Inspection - Attitude - Palmar subluxation and Ulnar deviation of the MCP joints; Swollen and Erythematous PIP joints; No swelling or redness of DIP joints; No apparent muscle wasting; Mild hyper-extension of PIP of thumbs; Pulp of fingers normal
-
Palpation - All Inspectory findings are confirmed;
Temperature raise +;
Wincing on gentle palpation of MCP joints and PIP joints; Palpation of DIP joints is normal; Swellings also + on 3rd and 4th PIP joints on both sides. Z-deformity +.
-
Range of Movements - Severe pain and severe limitation of active movements of flexion, extension and ulnar and radial deviation of MCP joints; severe pain and limitation of active and passive movements of flexion and extension at PIP joints. DIP joints normal.
Appendicular Skeleton -
Lower Limbs (Positive Findings only)
Hip Joints (both sides)
- Limitation of passive movements of flexion and extension (towards the end of range of motion);
Knee Joints (both sides)
-
Inspection - Swelling and erythema + ; Attitude - flexion;
-
Palpation - All Inspectory findings are confirmed; Raised temperature + ;
- Range of movements - Severe pain and limitation of active and passive movements of flexion and extension and lateral and medial rotation; (Patient was unable to stand on Day 1 and was able to stand on Day 2 with analgesic use).
Ankles (both sides)
-
Mild pain and limitation of active and passive movements of plantar flexion and dorsiflexion; Mild pain and limitation of movements of inversion and eversion.
-
Palpation of Achilles tendon is normal.
Foot examination (both sides)
- Mild pain and limitation of passive movements of flexion and extension of MTP joints; great toe flexion and extension normal;
Other Systems Examination
Cardiovascular System - No abnormalities detected
Respiratory System - No abnormalities detected
Abdominal Exam - No abnormalities detected
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